Erythema Elevatum Diutinum.
 

 

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Erythema Elevatum Diutinum./ Eritema Elevatum Diutinum  

Data-Medicos
Dermagic/Express No. 2-(84)
24 Noviembre 1.999. 24 November 1.999.

~ Eritema Elevatum Diutinum ~
~ Erythema Elevatum Diutinum ~

EDITORIAL ESPANOL
=================
Hola amigos DERMAGICOS, el tema de hoy, EL ERITEMA ELEVATUM DIUTINUM.
Descrito por primera vez en 1984 por Croker y William. Enfermedad poco
comun clasificada dentro de las dermatosis neutrofilicas como una
VASCULITIS LEUCOCITOCLASTICA ocasionada por deposision de complejos
inmunes a nivel vascular. Asociada a muchas otras enfermedades
DERMATOLOGICASY NO DERMATOLOGICAS como: pioderma gangrenoso, enfermedad
de Crohn, sindrome mielodisplastico, granuloma facial,
paraproteinemias, IgA monoclonal gammopatia, Wegener's granulomatosis,
SIDA, policondritis recidivante, artritis reumatoide, lupus eritematoso
sistemico, sindrome de overlap, hiperimmunoglobulinemia D sindrome,
enfermedad celiaca, etc. En estas 45 refrerencias los hechos.
En el atach eritema elevatum diutinum: palmas, codos y pies

Saludos a todos !!!

Dr. Jose Lapenta R.,,,

EDITORIAL ENGLISH
=================
Hello friends DERMAGICS, today's topic, THE ERYTHEMA ELEVATUM DIUTINUM.
Described for the first time in 1984 by Croker and William. Not very
common illness classified inside the neutrophilic diseases like a
LEUKOCYTOCLASTIC VASCULITIS caused by deposision of immune complexes at
vascular level. Associated to many other illnesses DERMATOLOGIC AND NON
DERMATOLOGIC like: pyoderma gangrenosum, Crohn's disease,
myelodisplastic syndrome, faciale granuloma, paraproteinemias, IgA
monoclonal gammopathy, Wegener's granulomatosis, acquired
immunodeficiency syndrome, relapsing polychondritis, rheumatoid
arthritis, systemic lupus erythematosus, averlap syndrome,
hyperimmunoglobulinaemia D syndrome, celiac disease etc.
In these 45 refrerencias the facts.


Greetings to ALL, !!
Dr. Jose Lapenta R.,,,
===================================================================
REFERENCIAS BIBLIOGRAFICAS / BIBLIOGRAPHICAL REFERENCES
===================================================================
============================================================
1.) [Erythema elevatum and diutinum in Crohn disease]
2.) Erythema elevatum diutinum associated with IgA paraproteinemia
successfully controlled with intermittent plasma exchange.
3.) Peripheral ulcerative keratitis associated with erythema elevatum
diutinum and a positive rheumatoid factor: a report of three cases.
4.) Terrien's marginal degeneration associated with erythema elevatum
diutinum.
5.) Erythema elevatum Diutinum.
6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms.
7.) Erythema elevatum diutinum: a clinicopathological study of eight
cases.
8.) Erythema elevatum diutinum with extensive acro-osteolysis.
9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory
reaction that occurs in settings other than erythema elevatum diutinum
and granuloma faciale.
10.) Erythema elevatum diutinum in association with coeliac disease.
11.) Erythema elevatum diutinum complicated by rheumatoid arthritis.
12.) Erythema elevatum diutinum in human immunodeficiency virus-infected

patients--report of a case and review of the literature [see comments]
13.) Unusual erythema elevatum diutinum with fibrohistiocytic
proliferation.
14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema
elevatum
diutinum, and rheumatoid arthritis as extramedullary manifestations of
plasma cell dyscrasia.
15.) A continuum of neutrophilic disease occurring in a patient with
ulcerative colitis.
16.) Granuloma faciale with extrafacial lesions.
17.) Erythema elevatum diutinum and HIV infection: a report of five
cases.
18.) Peripheral ulcerative keratitis associated with erythema elevatum
diutinum and a positive rheumatoid factor: a report of three cases.
19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory

reaction that occurs in settings other than erythema elevatum diutinum
and granuloma faciale.
20.) [Erythema elevatum diutinum. A rare dermatosis with a broad
spectrum of associated illnesses].
21.) Unusual erythema elevatum diutinum with fibrohistiocytic
proliferation.
22.) Erythema elevatum diutinum in human immunodeficiency virus-infected
patients--report of a case and review of the literature.
23.) Cutaneous vasculitis.
24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal
gammopathy.
25.) Simultaneous occurrence of two rare cutaneous markers of poor
prognosis in myelodysplastic syndrome: erythema elevatum diutinum and
specific lesions.
26.) Erythema elevatum diutinum: case report showing an unusual
distribution.
27.) Ultrastructural study of chronic lesions of erythema elevatum
diutinum: "extracellular cholesterosis" is a misnomer.
28.) Nodular lesions of erythema elevatum diutinum in patients infected
with the human immunodeficiency virus.
29.) Erythema elevatum diutinum associated with celiac disease: response
to a gluten-free diet.
30.) Association of hyperimmunoglobulinaemia D syndrome with erythema
elevatum diutinum.
31.) Erythema elevatum diutinum associated with Wegener's granulomatosis
and IgA paraproteinemia.
32.) Pyoderma gangrenosum in association with erythema elevatum
diutinum: report of two cases.
33.) Erythema elevatum diutinum: a clinicopathological study.
34.) Erythema elevatum diutinum in a patient with relapsing
polychondritis.
35.) Erythema elevatum diutinum: a clinical and histopathologic study of
13 patients.
36.) Erythema elevatum diutinum in a patient with acquired
immunodeficiency syndrome. Another clinical simulator of Kaposi's
sarcoma.
37.) Erythema elevatum diutinum--an unusual association with ulcerative
colitis.
38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda.
39.) Cutaneous manifestations of neutrophilic disease. A study of seven
cases.
40.) Erythema elevatum diutinum--a solitary lesion in a patient with
rheumatoid arthritis.
41.) Erythema elevatum diutinum in a patient with Crohn's disease.
42.) The natural history of vasculitis. What the histology tells us
about pathogenesis.
43.) Pleural effusions in an overlap syndrome of idiopathic
hypereosinophilic syndrome and erythema elevatum diutinum.
44.) Erythema elevatum diutinum: an ultrastructural case study.
45.) [Erythema elevatum diutinum--a rare variant of skin changes in
systemic lupus erythematosus].
============================================================
============================================================
1.) [Erythema elevatum and diutinum in Crohn disease]
============================================================
Author
Elsner J; Kiehl P; Kapp A; Weiss J
Address
Dermatologische Klinik und Poliklinik, Medizinische Hochschule Hannover.

Source
Hautarzt, 47(9):701-4 1996 Sep
Abstract
A 35-year-old woman suffering from Crohn's disease for 3 years developed

painful erythematous nodular lesions predominantly on the dorsa of her
hands and feet, as well as on her elbows and wrists. The clinical
features
and histopathological picture of leukocytoclastic vasculitis were both
highly suggestive of erythema elevatum diutinum. No improvement of the
skin
lesions was observed during treatment with high doses of oral steroids.
In
contrast, treatment with dapsone resulted in a marked reduction of skin
lesions. During a new attack of her Crohn's disease the erythema
elevatum
diutinum lesions flared again. The parallel clinical course of erythema
elevatum diutinum and Crohn's disease, as well as the detection of
immune
complexes and neutrophilic granulocytes in lesions of both diseases,
strongly suggest a common etiology. This hypothesis is discussed in
context
with this case and the recent literature.
============================================================
2.) Erythema elevatum diutinum associated with IgA paraproteinemia
successfully
controlled with intermittent plasma exchange.
============================================================
Author
Chow RK; Benny WB; Coupe RL; Dodd WA; Ongley RC
Address
Division of Dermatology, University of British Columbia, Vancouver.
Source
Arch Dermatol, 132(11):1360-4 1996 Nov
Abstract
BACKGROUND: We review the literature and report a case of refractory
erythema elevatum diutinum associated with IgA paraproteinemia that was
successfully controlled with intermittent plasma exchange (PLEX).
OBSERVATIONS: Typical lesions of erythema elevatum diutinum developed in
a
72-year-old patient with IgA paraproteinemia; the condition predictably
flared whenever IgA levels reached a threshold of 8 g/L. After 8 years
of
unsuccessful treatment with various agents, we instituted a trial of
PLEX
during an acute flare. Following 6 exchanges over a period of 2 weeks,
the
IgA level decreased from 8 to 2 g/L and the skin lesions cleared. Three
weeks later, new skin lesions developed and the IgA level had rebounded
from 2 to just over 8 g/L. A second course of PLEX was administered,
with
excellent results, and a 3-month course of oral chlorambucil (2 mg/d)
was
initiated. The patient's condition remained in clinical remission for 10

months. Over the ensuing 9 years, she suffered 11 further flares, each
of
which was associated with IgA levels of 8 to 10 g/L and each responding
dramatically to 3 to 5 PLEXs followed by a consolidative dose of
intravenous cyclophosphamide (250-500 mg). CONCLUSION: We believe that
PLEX
may have an important role in the management of severe erythema elevatum

diutinum associated with monoclonal paraproteinemia refractory to other

============================================================
3.) Peripheral ulcerative keratitis associated with erythema elevatum
diutinum and a positive rheumatoid factor: a report of three cases.
============================================================
Author
Takiwaki H; Kubo Y; Tsuda H; Arase S; Shiota H
Address
Department of Dermatology, School of Medicine, University of Tokushima,
Japan.
Source
Br J Dermatol, 138(5):893-7 1998 May
Abstract
Peripheral ulcerative keratitis (PUK) is a complication of
collagen-vascular diseases such as rheumatoid arthritis (RA) and other
systemic vasculitides. We report three cases of erythema elevatum
diutinum
with PUK. These patients presented with nodules and plaques consistent
with
erythema elevatum diutinum on the extremities and crusted or ulcerated
purpuric lesions on the soles. Histopathological examination of these
lesions revealed a dense neutrophilic infiltrate with nuclear dust and
fibrin around blood vessels. All the patients developed PUK concomitant
with the development of the skin lesions. The rheumatoid factor was
positive at high titre in all three patients; this was associated with
probable RA in one. Cutaneous lesions were dramatically improved by
administration of dapsone in all cases. Dapsone was also effective in
treating the ocular lesions in two patients.

============================================================
4.) Terrien's marginal degeneration associated with erythema elevatum
diutinum.
============================================================
Author
Shimazaki J; Yang HY; Shimmura S; Tsubota K
Address
Department of Ophthalmology, Tokyo Dental College, Chiba, Japan.
Source
Cornea, 17(3):342-4 1998 May
Abstract
PURPOSE: To describe a patient with Terrien's marginal degeneration
associated with a chronic skin disease, erythema elevatum diutinum.
METHODS: A 27-year-old woman with peripheral thinning of her left cornea

associated with adjacent conjunctival and episcleral inflammation was
clinically examined. RESULTS: Episodes of worsening skin lesions were
associated with development of inflammation in her left eye.
Administration
of sulfones was effective for reducing both skin and ocular
inflammation.
CONCLUSION: Erythema elevatum diutinum should be considered an
underlying
systemic disease of Terrien's marginal degeneration.
============================================================
5.) Erythema elevatum diutinum.
============================================================
Author:
Croker H W, Williams C.
Br J Dermatol 1984;6:1-9,33,8
============================================================
============================================================
6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms.
Author
============================================================
Shanks JH; Banerjee SS; Bishop PW; Pearson JM; Eyden BP
Address
Department of Histopathology, Christie Hospital NHS Trust, UK.
Source
Histopathology, 31(1):91-6 1997 Jul
Abstract
AIMS: We describe the cutaneous pseudoneoplastic lesions in two patients

with nodular erythema elevatum diutinum, a rare chronic disorder in
which
polymorph nuclear fragmentation (leukocytoclasis) is present within
dermal
nodules showing spindle cells and fibrosis. In both cases diagnostic
difficulty was encountered clinically and pathologically and various
benign
and malignant neoplasms were considered in the differential diagnosis.
METHODS AND RESULTS: Immunohistochemically the spindle cells were
negative
for CAM5.2, AE1/3, S100 protein and desmin (D33). They were positive for

vimentin and focally positive for CD34 and alpha-smooth muscle actin.
Some
of the spindle cells were positive for Mac 387 and KP1(CD68). By
electron
microscopy, the lesions were shown to consist of
fibroblasts/myofibroblasts
and fusiform macrophages. CONCLUSIONS: Increased awareness of the
features
described will help to avoid misdiagnosis as a neoplastic process.

============================================================
7.) Erythema elevatum diutinum: a clinicopathological study of eight
cases.
Author
============================================================
Sang¨ueza OP; Pilcher B; Martin Sang¨ueza J
Address
Department of Pathology, Medical College of Georgia, Augusta 30912, USA.

Source
Am J Dermatopathol, 19(3):214-22 1997 Jun
Abstract
Erythema elevatum diutinum (EED) is a rare cutaneous condition that
initially presents as leukocytoclastic vasculitis (LCCV) of the skin and

later resolves with fibrosis. In addition to the LCCV, EED may show
features reminiscent of other entities. For example, it may mimic
lesions
of dermatofibroma, granuloma annulare, granuloma faciale, or dermatitis
herpetiformis. For this study, we reviewed the clinical records and 13
skin
biopsies in eight patients with EED. One of the patients had concurrent
pityriasis rubra pilaris, and another developed lesions of EED following

and at the sites of mosquito bites; these associations have not been
noted
previously. In addition to such typical histopathological features as
diffuse dermal involvement by neutrophils, eosinophils, and
leukocytoclastic vasculitis, we also found two unusual patterns. The
first
was characterized by palisaded necrotizing granulomas, as previously
described and associated with Churg-Strauss granuloma; the second
condition
simulated a pyogenicgranuloma--like lesion.

============================================================
8.) Erythema elevatum diutinum with extensive acro-osteolysis.
Author
============================================================
Ellabban A; Schumacher HR Jr
Address
Division of Rheumatology, University of Pennsylvania School of Medicine,

Philadelphia, USA.
Source
J Rheumatol, 24(6):1203-5 1997 Jun
Abstract
Erythema elevatum diutinum (EED) is a rare chronic skin disease with
cutaneous vasculitis, characterized by the absence of systemic
vasculopathy. We describe a 79-year-old white woman who has been
followed
with the diagnosis of EED for 27 years. Our patient has a previously
unreported combination of EED and acro-osteolysis. After reviewing
possible
reasons for the association, her vasculitis seems the most likely
factor.

============================================================
9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory
reaction that occurs in settings other than erythema elevatum diutinum
and
granuloma faciale.
============================================================
Author
Carlson JA; LeBoit PE
Address
Department of Pathology, Albany Medical College, New York, USA.
Source
Am J Surg Pathol, 21(6):698-705 1997 Jun
Abstract
Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic,

localized forms of cutaneous leukocytoclastic vasculitis that result in
patterned (storiform or concentric) fibrosis. EED often occurs in
systemically ill patients as bilaterally symmetrical plaques, papules,
or
nodules, often over the dorsa of joints. GF occurs as one or a few
plaques
on the face. Eosinophils and plasma cells are prominent in GF, whereas
neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany
facial ones in GF, and there are a few reports of upper respiratory
tract
masses with GF-like histologic features. We report on eight patients
with
solitary cutaneous lesions with histologic features similar to those of
EED
or GF, but whose clinical picture was not that of either disease. One,
whose histology resembled GF, had a large multinodular dermal and
subcutaneous mass that persisted despite attempted resection. Unusual
histologic findings in other cases included storiform fibrosis with
dense
infiltrates of plasma cells, branching nerve fascicles admixed with
EED-like changes, and EED-like areas adjacent to zones mimicking a
sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the
stereotypic settings of GF and EED and is an inflammatory reaction
pattern
that does not signify a specific diagnosis. Because of transitions
between
EED or GF-like areas and those of patterned sclerosis with plasma
cell-rich
infiltrates, we believe that some "inflammatory pseudotumors" of the
skin,
and perhaps of other sites could be the result of localized vasculitis.

============================================================
10.) Erythema elevatum diutinum in association with coeliac disease.
Author
============================================================
Tasanen K; Raudasoja R; Kallioinen M; Ranki A
Address
Department of Dermatology, Oulu University Hospital, Finland.
Source
Br J Dermatol, 136(4):624-7 1997 Apr
Abstract
Erythema elevatum diutinum (EED) has been described in association with
several immunological or infectious diseases. We describe a female
patient
who presented with clinically and histologically typical EED in whom
previously undiagnosed coeliac disease was found. Appearance of EED
lesions
was preceded by widespread joint pains. In extensive laboratory tests,
the
only abnormal findings were an elevated erythrocyte sedimentation rate
(ESR) and decreased haemoglobin and folic acid levels. Later, IgA and
IgG
type antireticulin and antigliadin antibodies were detected. Serum total

IgA was elevated but no paraproteinaemia was found. In lesional skin,
granular deposits of IgA and C3 were seen at the dermo-epidermal
junction.
A duodenal biopsy revealed total villous atrophy. Dapsone treatment was
partly effective but complete healing of the EED lesions was achieved
only
after the introduction of a strict gluten-free diet. The patient has now

remained symptom-free on the diet for 1.5 years.

============================================================
11.) Erythema elevatum diutinum complicated by rheumatoid arthritis.
============================================================
Author
Nakajima H; Ikeda M; Yamamoto Y; Kodama H
Address
Department of Dermatology, Kochi Medical School, Japan.
Source
J Dermatol, 26(7):452-6 1999 Jul
Abstract
A 53-year-old female developed erythema elevatum diutinum (EED) twelve
years after the onset of rheumatoid arthritis. The arthritis had been
well
controlled for the last several years. Annular purpuric macules were
characteristically complicated by common nodular and plaque lesions.
Both
leukocytoclastic vasculitis and fibrosis were observed in the macular
lesions, indicating that the lesions were a manifestation of an early
phase
of EED. Both types of skin lesions disappeared with treatment with
dapsone.
They have not relapsed for two years after stopping the dapsone. The
leukocytoclastic vasculitis was thought to have developed independently
of
the rheumatoid arthritis. She had noticed sicca symptoms two years
before
the appearance of EED, but she did not satisfy the diagnostic criteria
for
Sj¨ogren's syndrome.

============================================================
12.) Erythema elevatum diutinum in human immunodeficiency virus-infected

patients--report of a case and review of the literature [see comments]
============================================================
Author
Dronda F; Gonz´alez-L´opez A; Lecona M; Barros C
Address
Hospital General Penitenciario, Madrid, Spain.
Source
Clin Exp Dermatol, 21(3):222-5 1996 May
Abstract
Erythema elevatum diutinum (EED) is a rare chronic disease of unknown
origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A

case of EED in a 32-year-old HIV-infected male patient, with no previous

opportunistic infections and a CD4+ cell count of less than 200/mm3, is
reported. Therapy with oral dapsone (100 mg/day for 15 days) resulted in

clinical cure with no relapse after 6 months of follow-up. To our
knowledge, only six cases of EED in HIV-positive patients have been
reported to date. A brief review of these seven cases is described.

============================================================
13.) Unusual erythema elevatum diutinum with fibrohistiocytic
proliferation.
============================================================
Author
Porneuf M; Duterque M; Sotto A; Jourdan J
Address
Department of Internal Medicine, University of Nimes, France.
Source
Br J Dermatol, 134(6):1131-4 1996 Jun
Abstract
A 78-year-old woman with a history of symmetrical erythematous plaques
on
the arms, and a monoclonal gammopathy, developed a strange striped
reticulate papular dermatosis with central atrophy. Histological
examination was compatible with a very late stage of erythema elevatum
diutinum (EED), showing a fibrohistiocytic proliferation with areas of
granulation tissue. This fibrosis may result from the chronic dermal
injury
of leucocytoclastic vasculitis and is sometimes the predominant
histology
of EED. Investigations for underlying haematological anomalies, such as
paraproteinaemia and infection with human immunodeficiency virus, must
be
performed. Dapsone is ineffective once the fibrous nodules have
appeared.

============================================================
14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema
elevatum
diutinum, and rheumatoid arthritis as extramedullary manifestations of
plasma cell dyscrasia.
============================================================
Author
Albitar S; Bourgeon B; Genin R; Jacquesson M; Riviere JP; Serveaux MO;
Ribera A; Serveaux JP
Address
Hemodialysis Center, AURAR, 6 Avenue Stanislas Gimart, F-97490 Ste
Clotilde
de La Reunion, France.
Source
Am J Kidney Dis, 31(4):E3 1998 Apr
Abstract
POEMS syndrome is a rare synopsis of different multisystemic disorders
(polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy,
and
skin lesions) associated with plasma cell dyscrasia. We herein report
the
atypical case of a 44-year-old white man presenting with glomerulopathy,

POEMS syndrome, and erythema elevatum diutinum with a few-year history
of
non-insulin-dependent diabetes mellitus (NIDDM) and seronegative
rheumatoid
arthritis (RA) as early manifestations of IgAlambda multiple myeloma.
The
prescription of 1 mg/kg/day prednisone improved the patient's features
dramatically. Skin lesions improved by the association of
glucocorticoids
and plasma exchange, recurred when plasmapheresis ceased, and remitted
when
plasma exchange was reintroduced. NIDDM requiring insulinotherapy
recurred
when corticoids were discontinued and remitted when prednisone was
reintroduced. However, prednisone and plasmapheresis had no effect on
polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient,

who developed indolent multiple myeloma a few years later. We thus
concluded that POEMS syndrome, steroid-dependent diabetes mellitus,
rheumatoid arthritis, RA, and skin vasculitis in our patient were
triggered
by plasma cell dyscrasia.

============================================================
15.) A continuum of neutrophilic disease occurring in a patient with
ulcerative
colitis.
============================================================
Author
Salmon P; Rademaker M; Edwards L
Address
Department of Dermatology, Waikato Hospital, Health Waikato, Hamilton,
New
Zealand.
Source
Australas J Dermatol, 39(2):116-8 1998 May
Abstract
A case of Sweet's syndrome (acute febrile neutrophilic dermatosis)
occurring concurrently with bullous pyoderma gangrenosum is reported to
emphasize the close relationship between these two disorders. Atypical
pyoderma gangrenosum and Sweet's syndrome have been described as
occurring
simultaneously in haematological dyscrasias but not, to our knowledge,
in
ulcerative colitis. It has been proposed that pyoderma gangrenosum,
Sweet's
syndrome, erythema elevatum diutinum and subcorneal pustular dermatosis
may
represent manifestations along a continuum of neutrophilic dermatoses.

============================================================
16.) Granuloma faciale with extrafacial lesions.
============================================================
Author
Roustan G; S´anchez Yus E; Salas C; Sim´on A
Address
Department of Dermatology, Cl´inica Universitaria Puerta de Hierro,
Madrid,
Espa~na.
Source
Dermatology, 198(1):79-82 1999
Abstract
Extrafacial involvement in granuloma faciale (GF) is rather exceptional.
We
report herein a patient with GF associated with lesions on the trunk and

the forearm. Histological studies of facial and extrafacial lesions
shared
similar characteristics: a mixed inflammatory infiltrate with abundant
eosinophils in the superficial and middle dermis with a narrow grenz
zone
of uninvolved dermis between the epidermis and the infiltrate. Evidence
of
vasculitis was clearly observed in both biopsy specimens. Treatment with

dapsone did not alter the course of the disease. We review the 12 cases
of
extrafacial GF that have been reported in the English and Spanish
literature. In these cases a diagnosis of erythema elevatum diutinum
(EED)
may be suggested. Although GF and EED may share some pathogenic
mechanisms,
there are several clinical and histological differences between them
that
make us consider EED and GF as distinct entities.

============================================================
17.) Erythema elevatum diutinum and HIV infection: a report of five
cases.
============================================================
Br J Dermatol 1999 Aug;141(2):335-338

Muratori S, Carrera C, Gorani A, Alessi E
Institute of Dermatological Sciences, University of Milan, IRCCS
Ospedale
Maggiore, Via Pace 9, 20122 Milan, Italy.


Erythema elevatum diutinum (EED) is emerging as a specific
HIV-associated
dermatosis, 11 cases having so far been reported in the medical
literature
and five patients with the disease having been seen by us during the
last 4
years. As the disease is poorly known, it is easily confused with
Kaposi's
sarcoma or bacillary angiomatosis, but the histopathological features
are
diagnostic. EED is considered to be an immune complex-mediated
vasculitis.
A streptococcal infection seemed to be the trigger factor in four of our

patients. Partial control of the cutaneous lesions was achieved by the
use
of antibiotics.

============================================================
18.) Peripheral ulcerative keratitis associated with erythema elevatum
diutinum
and a positive rheumatoid factor: a report of three cases.
============================================================
Br J Dermatol 1998 May;138(5):893-7

Takiwaki H, Kubo Y, Tsuda H, Arase S, Shiota H
Department of Dermatology, School of Medicine, University of Tokushima,
Japan.

Peripheral ulcerative keratitis (PUK) is a complication of
collagen-vascular diseases such as rheumatoid arthritis (RA) and other
systemic vasculitides. We report three cases of erythema elevatum
diutinum
with PUK. These patients presented with nodules and plaques consistent
with
erythema elevatum diutinum on the extremities and crusted or ulcerated
purpuric lesions on the soles. Histopathological examination of these
lesions revealed a dense neutrophilic infiltrate with nuclear dust and
fibrin around blood vessels. All the patients developed PUK concomitant
with the development of the skin lesions. The rheumatoid factor was
positive at high titre in all three patients; this was associated with
probable RA in one. Cutaneous lesions were dramatically improved by
administration of dapsone in all cases. Dapsone was also effective in
treating the ocular lesions in two patients.

============================================================
19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory

reaction that occurs in settings other than erythema elevatum diutinum
and
granuloma faciale.
============================================================
Am J Surg Pathol 1997 Jun;21(6):698-705

Carlson JA, LeBoit PE
Department of Pathology, Albany Medical College, New York, USA.

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic,

localized forms of cutaneous leukocytoclastic vasculitis that result in
patterned (storiform or concentric) fibrosis. EED often occurs in
systemically ill patients as bilaterally symmetrical plaques, papules,
or
nodules, often over the dorsa of joints. GF occurs as one or a few
plaques
on the face. Eosinophils and plasma cells are prominent in GF, whereas
neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany
facial ones in GF, and there are a few reports of upper respiratory
tract
masses with GF-like histologic features. We report on eight patients
with
solitary cutaneous lesions with histologic features similar to those of
EED
or GF, but whose clinical picture was not that of either disease. One,
whose histology resembled GF, had a large multinodular dermal and
subcutaneous mass that persisted despite attempted resection. Unusual
histologic findings in other cases included storiform fibrosis with
dense
infiltrates of plasma cells, branching nerve fascicles admixed with
EED-like changes, and EED-like areas adjacent to zones mimicking a
sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the
stereotypic settings of GF and EED and is an inflammatory reaction
pattern
that does not signify a specific diagnosis. Because of transitions
between
EED or GF-like areas and those of patterned sclerosis with plasma
cell-rich
infiltrates, we believe that some "inflammatory pseudotumors" of the
skin,
and perhaps of other sites could be the result of localized vasculitis.

============================================================
20.) [Erythema elevatum diutinum. A rare dermatosis with a broad
spectrum of
associated illnesses].
============================================================
Hautarzt 1997 Feb;48(2):113-7

Gerbig AW, Zala L, Hunziker T
Dermatologische Universitatsklinik, Bern.

Erythema elevatum diutinum (EED) is a rare disease presenting with
persistent red to yellow-brown papules or plaques which are mainly
localized symmetrically on the extensor aspects of the hands and
fingers,
the elbows and the knees. The histology shows a leucocytoclastic
vasculitis
in early lesions and fibrosis of the dermis later on. Dapsone is the
treatment of choice. Today, EED is usually assigned to the neutrophilic
dermatoses in which an association with hematological disturbances is
well
documented. We report on a patient with EED and glioma WHO grade IV, a
coincidence unreported hitherto. Additionally, we review the literature
on
diseases associated with EED.

============================================================
21.) Unusual erythema elevatum diutinum with fibrohistiocytic
proliferation.
============================================================
Br J Dermatol 1996 Jun;134(6):1131-4

Porneuf M, Duterque M, Sotto A, Jourdan J
Department of Internal Medicine, University of Nimes, France.

A 78-year-old woman with a history of symmetrical erythematous plaques
on
the arms, and a monoclonal gammopathy, developed a strange striped
reticulate papular dermatosis with central atrophy. Histological
examination was compatible with a very late stage of erythema elevatum
diutinum (EED), showing a fibrohistiocytic proliferation with areas of
granulation tissue. This fibrosis may result from the chronic dermal
injury
of leucocytoclastic vasculitis and is sometimes the predominant
histology
of EED. Investigations for underlying haematological anomalies, such as
paraproteinaemia and infection with human immunodeficiency virus, must
be
performed. Dapsone is ineffective once the fibrous nodules have
appeared.

============================================================
22.) Erythema elevatum diutinum in human immunodeficiency virus-infected

patients--report of a case and review of the literature.
============================================================
Clin Exp Dermatol 1996 May;21(3):222-5

Dronda F, Gonzalez-Lopez A, Lecona M, Barros C
Hospital General Penitenciario, Madrid, Spain.

Erythema elevatum diutinum (EED) is a rare chronic disease of unknown
origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A

case of EED in a 32-year-old HIV-infected male patient, with no previous

opportunistic infections and a CD4+ cell count of less than 200/mm3, is
reported. Therapy with oral dapsone (100 mg/day for 15 days) resulted in

clinical cure with no relapse after 6 months of follow-up. To our
knowledge, only six cases of EED in HIV-positive patients have been
reported to date. A brief review of these seven cases is described.

============================================================
23.) Cutaneous vasculitis.
============================================================
Rheum Dis Clin North Am 1995 Nov;21(4):1097-113

Gibson LE, Su WP
Mayo Clinic, Rochester, Minnesota, USA.

Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and

histopathologic findings that share the common theme of vascular
inflammation and blood vessel damage. Clinical pattern and proper
histopathologic studies are essential to the proper classification and
evaluation of vasculitis. This article discusses the major types of
cutaneous vasculitis typified by necrotizing or leukocytoclastic
vasculitis
and includes discussions of Henoch-Schonlein purpura, urticarial
vasculitis, erythema elevatum diutinum, cutaneous polyarteritis nodosa,
livedoid vasculitis, and cutaneous granulomatous vasculitis.

============================================================
24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal

gammopathy.
============================================================
Australas J Dermatol 1995 Feb;36(1):21-3

Wayte JA, Rogers S, Powell FC
Regional Centre of Dermatology, Mater Misericordiae Hospital, Dublin,
Ireland.

A patient with erythema elevatum diutinum (EED) developed pyoderma
gangrenosum (PG). Investigation revealed an IgA kappa monoclonal
gammopathy. Previous reports of PG in association with EED are reviewed
and
the spectrum of the neutrophilic dermatoses discussed.

============================================================
25.) Simultaneous occurrence of two rare cutaneous markers of poor
prognosis in myelodysplastic syndrome: erythema elevatum diutinum and
specific lesions.
============================================================
Br J Dermatol 1994 Jul;131(1):112-7

Aractingi S, Bachmeyer C, Dombret H, Vignon-Pennamen D, Degos L,
Dubertret L
Department of Dermatology, H pital Saint-Louis, Paris, France.

We report the concomitant occurrence of erythema elevatum diutinum and
specific skin lesions in a patient with a myelodysplastic syndrome
(MDS).
This patient's course, and review of other reported cases, support the
opinion that neutrophilic dermatoses are associated with a poor
prognosis
of MDS. The simultaneous appearance of these manifestations could be the

consequence of a particular chemotactism of myeloid cells, expressed
after
acute transformation.

============================================================
26.) Erythema elevatum diutinum: case report showing an unusual
distribution.
============================================================
Cutis 1994 Mar;53(3):124-6

Hansen U, Haerslev T, Knudsen B, Jacobsen GK
Department of Pathology, Gentofte Hospital, University of Copenhagen,
Denmark.

A case of erythema elevatum diutinum showing an unusual distribution is
reported. Hard nodules were located on the patient's fingertips and
pulps
while the extensor surfaces were not involved. The disease was initially

misinterpreted based on both clinical and histologic examination.
Dapsone
therapy was followed by a remarkable recovery. The clinical and
histologic
features of erythema elevatum diutinum are summarized.

============================================================
27.) Ultrastructural study of chronic lesions of erythema elevatum
diutinum:
"extracellular cholesterosis" is a misnomer.
============================================================
J Am Acad Dermatol 1993 Aug;29(2 Pt 2):363-7

Kanitakis J, Cozzani E, Lyonnet S, Thivolet J
Department of Dermatology, Ed. Herriot Hospital, Lyon, France.

Erythema elevatum diutinum (EED) is a rare disease of unknown origin
that
belongs to the spectrum of leukocytoclastic vasculitis. Chronic lesions
of
EED contain lipid deposits, for which the term extracellular
cholesterosis
has been coined. We studied a typical case of EED with long-standing
lesions. Findings of electron microscopic examination revealed a heavy,
exclusively intracellular lipid deposition that consisted of lipid
droplets, myelin figures, and rare cholesterol clefts within histiocytes

but also within epidermal keratinocytes, mast cells, pericytes, and
lymphocytes. These findings are in keeping with the results of previous
ultrastructural studies and suggest that the term extracellular
cholesterosis is a misnomer; intracellular lipidosis would more
accurately
describe the lipid deposition.

============================================================
28.) Nodular lesions of erythema elevatum diutinum in patients infected
with the human immunodeficiency virus.
============================================================
J Am Acad Dermatol 1993 Jun;28(6):919-22

LeBoit PE, Cockerell CJ
Department of Pathology, School of Medicine, University of California,
San
Francisco 94143-0506.

BACKGROUND: Erythema elevatum diutinum (EED) is a rare, chronic form of
cutaneous vasculitis that can result in fibrosis. Four patients infected

with the human immunodeficiency virus (HIV) had unusual nodular lesions
of
EED. OBJECTIVE: Our purpose was to document the occurrence of these
lesions
in HIV-infected patients and to characterize the histopathologic and
immunophenotypic features of nodular EED. METHODS: Clinicopathologic
studies were supplemented by special stains of biopsy specimens.
RESULTS:
EED may be a complication of HIV infection. Nodules of EED are composed
of
small aggregates of Mac-387+ spindled cells. Within the aggregates can
be
seen neutrophils, nuclear dust, and fibrin. The marked fibrosis of the
lesions may account for the lack of response to dapsone. CONCLUSION: EED

should be considered in the differential diagnosis of juxta-articular
nodules in HIV-infected patients; its diagnosis can be confirmed by
biopsy.

============================================================
29.) Erythema elevatum diutinum associated with celiac disease: response
to a gluten-free diet.
============================================================
Pediatr Dermatol 1993 Jun;10(2):125-8

Rodriguez-Serna M, Fortea JM, Perez A, Febrer I, Ribes C, Aliaga A
Department of Dermatology, Hospital General, Valencia, Spain.

An 11-year-old girl with a history of insulin-dependent diabetes
mellitus
had erythema elevatum diutinum (EED) associated with a celiac disease
related to a possible kidney disease. Dapsone did not improve the skin
manifestations. However, the lesions disappeared after a gluten-free
diet
was begun. To our knowledge, this report describes the first case of EED
in
a patient with celiac disease.

============================================================
30.) Association of hyperimmunoglobulinaemia D syndrome with erythema
elevatum diutinum.
============================================================
Br J Dermatol 1993 May;128(5):572-4

Miyagawa S, Kitamura W, Morita K, Saishin M, Shirai T
Department of Dermatology, Nara Medical University, Japan.

Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a

female patient with erythema elevatum diutinum. The association of this
skin disease with hyperimmunoglobulinaemia D may indicate a pathogenetic

relationship. The skin lesions responded to dapsone therapy.

============================================================
31.) Erythema elevatum diutinum associated with Wegener's granulomatosis
and IgA paraproteinemia.
============================================================
J Am Acad Dermatol 1993 May;28(5 Pt 2):846-9

Kavanagh GM, Colaco CB, Bradfield JW, Archer CB
Department of Dermatology, Bristol Royal Infirmary, University of
Bristol,
United Kingdom.

A 69-year-old man had erythema elevatum diutinum for several years
before
he developed IgA paraproteinemia and a limited form of Wegener's
granulomatosis. This is the first report of an association between
erythema
elevatum diutinum and Wegener's granulomatosis. IgA paraproteinemia has
been reported in association with erythema elevatum diutinum but not
with
Wegener's granulomatosis.

============================================================
32.) Pyoderma gangrenosum in association with erythema elevatum
diutinum: report of two cases.
============================================================
Cutis 1992 Mar;49(3):261-6

Planaguma M, Puig L, Alomar A, Matias-Guiu X, de Moragas JM
Department of Dermatology, Hospital de la Santa Creu i Sant Pau,
Autonomous
University of Barcelona, Spain.

We report on the appearance of centrifugally spreading ulcers with
undermined borders in two patients with chronic recurrent erythema
elevatum
diutinum controlled with dapsone. The ulcerated lesions were consistent
on
clinical and pathologic examination with the diagnosis of pyoderma
gangrenosum. They eventually responded to treatment with oral
corticosteroids. The addition of cyclosporine was required in one case.
No
associated disease was found in any of the patients. The possible
pathophysiological mechanisms of this uncommon association are reviewed.

============================================================
33.) Erythema elevatum diutinum: a clinicopathological study.
============================================================
Clin Exp Dermatol 1992 Mar;17(2):87-93

Wilkinson SM, English JS, Smith NP, Wilson-Jones E, Winkelmann RK
Department of Dermatology, North Staffordshire Hospital Centre,
Stoke-on-Trent, U.K.

Erythema elevatum diutinum is a syndrome of vasculitis in which lesions,

typically over the extensor surfaces, showed a mixed inflammatory
infiltrate on biopsy. We describe a series of 13 patients. The most
common
association in our series was with hypergammaglobulinemia; both mono and

polyclonal. Chronic infection, not streptococcal, was a less frequent
finding although two of three patients had a positive reaction to the
intradermal injection of streptococcal antigen. Dapsone remains the
initial
treatment of choice.

============================================================
34.) Erythema elevatum diutinum in a patient with relapsing
polychondritis.
============================================================
J Am Acad Dermatol 1992 Feb;26(2 Pt 2):312-5

Bernard P, Bedane C, Delrous JL, Catanzano G, Bonnetblanc JM
Department of Dermatology, CHRU Dupuytren, Limoges, France.

A 69-year-old man with a history of relapsing polychondritis developed a

widespread cutaneous eruption that was clinically and histologically
typical of erythema elevatum diutinum. This eruption was accompanied by
a
recurrence of arthralgias. The significance of this association between
relapsing polychondritis and erythema elevatum diutinum, which has not
been
previously reported, is discussed.

============================================================
35.) Erythema elevatum diutinum: a clinical and histopathologic study of
13
patients.
============================================================
J Am Acad Dermatol 1992 Jan;26(1):38-44

Yiannias JA, el-Azhary RA, Gibson LE
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.

BACKGROUND: Erythema elevatum diutinum is a rare condition representing
a
chronic leukocytoclastic vasculitis. OBJECTIVE: Clinical and laboratory
features of the disease were reviewed to better understand the disease.
METHODS: The medical records and histopathologic slides of 13 patients
with
erythema elevatum diutinum were studied. RESULTS: The lesions were
violaceous, deep red, or brown and typically were papules or plaques.
Lesions were most often located on the extensor surfaces of the
extremities. Associated medical problems included hematologic
abnormalities
in six patients: IgA clonal gammopathies (four), multiple myeloma (one),

and myelodysplasia (one). Erythema elevatum diutinum preceded the
myeloproliferative disorders by an average of 7.8 years. All patients
showed vasculitis. Leukocytoclasia was present in 27 of 35 specimens.
The
predominant cell type in the inflammatory infiltrate was
polymorphonuclear
leukocytes or a mixture of polymorphonuclear leukocytes and lymphocytes.

CONCLUSION: The most significant finding of this study is the
association
of erythema elevatum diutinum with hematologic disease, most frequently
an
IgA monoclonal gammopathy.

============================================================
36.) Erythema elevatum diutinum in a patient with acquired
immunodeficiency
syndrome. Another clinical simulator of Kaposi's sarcoma.
============================================================
Arch Dermatol 1991 Dec;127(12):1819-22

Requena L, Sanchez Yus E, Martin L, Barat A, Arias D
Department of Dermatology, Jimenez Diaz Foundation, Nuestra Senora de la

Concepcion Clinic, Universidad Autonoma, Madrid, Spain.

Several types of vasculitis have been described in patients with human
immunodeficiency virus infection. Erythema elevatum diutinum is a rare
variant of cutaneous leukocytoclastic vasculitis which, with the
exception
of the case reported herein, has been described only once in human
immunodeficiency virus-infected patients. Our male patient, a longtime
intravenous drug abuser, had cutaneous lesions, closely resembling
Kaposi's
sarcoma, on the extensor surfaces of the lower extremities. Cutaneous
biopsy specimens, however, demonstrated leukocytoclastic vasculitis with

fibrinoid necrosis of the vessel walls and areas of basophilic
degeneration
of collagen bundles in early lesions, whereas late lesions showed dense
diffuse fibrosis with proliferation of dermal spindle cells and some
foci
of residual leukocytoclastic vasculitis. Oral therapy with dapsone
resulted
in marked clearing of the cutaneous lesions within few days. This case
raises the necessity of histologic confirmation for all cases of
suspected
Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. We

discuss the possible pathogenesis of leukocytoclastic vasculitis in
human
immunodeficiency virus-infected patients.

============================================================
37.) Erythema elevatum diutinum--an unusual association with ulcerative
colitis.
============================================================
Clin Exp Dermatol 1991 May;16(3):204-6

Buahene K, Hudson M, Mowat A, Smart L, Ormerod AD
Department of Dermatology, Aberdeen Royal Infirmary, UK.

We report the association of erythema elevatum diutinum (EED) with
ulcerative colitis (UC). The patient, whose history included rheumatoid
disease, diabetes mellitus (type I) and hypothyroidism developed EED
during
a severe acute exacerbation of UC which resolved following colectomy. To

our knowledge this is the first report of such an association. Also of
interest was the observation of Koebner phenomenon occurring at the
sites
where blood vessels were damaged.

============================================================
38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda.
============================================================
Br J Dermatol 1991 Jan;124(1):89-91

Requena L, Barat A, Hasson A, Arias D, Gutierrez MC, Martin L, De Castro
A
Department of Dermatology, Universidad Autonoma, Madrid, Spain.

A case of erythema elevatum diutinum (EED) closely resembling porphyria
cutanea tarda (PCT) is reported. The initial skin biopsies were
suggestive
for PCT but porphyrin levels in the urine, stool and plasma were normal.
A
further biopsy from an early cutaneous lesion showed a leucocytoclastic
vasculitis with fibrinoid necrosis of the vessel walls.

============================================================
39.) Cutaneous manifestations of neutrophilic disease. A study of seven
cases.
============================================================
Dermatologica 1991;183(4):255-64

Vignon-Pennamen MD, Wallach D
Department of Dermatology, Hopital Saint-Louis, Paris, France.

Seven patients with a complex form of neutrophilic dermatosis are
reported.
Clinically, they had variable associations of four types of lesions:
blisters/pustules, plaques, nodules and ulcerations. Histologically, a
neutrophilic infiltrate was observed at variable levels in the
epidermis,
dermis and subcutis. Systemic manifestations were present in all cases
(general symptoms, joint, renal, ocular and lung involvements). Three
patients had an associated disease (myelodysplasia, metastatic
carcinoma,
IgG gammopathy). Steroids were the most efficient treatment. These
observations, as well as a review of the literature, support the opinion

that the neutrophilic dermatosis represents a continuous spectrum
encompassing four well-defined entities: subcorneal pustular dermatosis,

Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum.
We
propose that the different patterns of the neutrophilic dermatosis are
the
most obvious manifestations of a potentially multisystemic neutrophilic
disease and allow its recognition.

============================================================
40.) Erythema elevatum diutinum--a solitary lesion in a patient with
rheumatoid arthritis.
============================================================
Clin Exp Dermatol 1990 Sep;15(5):394-5

Collier PM, Neill SM, Branfoot AC, Staughton RC
Department of Dermatology, Westminster Hospital, London, UK.

Erythema elevatum diutinum is a rare disease of unknown aetiology. It is

usually symmetrical with multiple lesions. An association with
rheumatoid
has previously been reported. We report a case of atypical erythema
elevatum diutinum affecting the right elbow of a female patient with
seropositive rheumatoid arthritis.

============================================================
41.) Erythema elevatum diutinum in a patient with Crohn's disease.
============================================================
J Am Acad Dermatol 1990 May;22(5 Pt 2):948-52

Walker KD, Badame AJ
Department of Dermatology, Tulane University Medical School, New
Orleans,
LA 70112.

A possible association between erythema elevatum diutinum and Crohn's
disease is described. To our knowledge, erythema elevation diutinum has
never been reported in a patient with Crohn's disease, nor has the
disorder
been considered one of the cutaneous manifestations of inflammatory
bowel
disease. Erythema elevatum diutinum is believed to be mediated by the
deposition of circulating immune complexes in the dermal perivascular
spaces. Our patient showed clinical and histopathologic findings typical
of
both erythema elevatum diutinum and Crohn's disease. The patient also
had a
positive Raji cell assay, which demonstrates the presence of a
circulating
immune complex. Increased levels of circulating immune complexes have
been
found in patients with inflammatory bowel disease, and the
gastrointestinal
inflammation in Crohn's disease could lead to enhanced antigenic
exposure
and increased formation of immune complexes. We contend that
inflammatory
bowel disease may have contributed to the development of erythema
elevatum
diutinum in our patient.

============================================================
42.) The natural history of vasculitis. What the histology tells us
about
pathogenesis.
============================================================
Arch Dermatol 1990 Jan;126(1):84-9

Smoller BR, McNutt NS, Contreras F
Department of Medicine (Dermatology), New York Hospital-Cornell
University
Medical Center, NY 10021.

While histopathologic analysis may offer some clues as to the
pathogenesis
of vasculitis, observations must be interpreted with caution, as there
is
considerable overlap in the histologic pattern. In most cases, a
predominantly neutrophilic vasculitis affecting small dermal venules
suggests a relatively acute, immune complex-mediated reaction. Less
commonly, this histologic pattern may be seen in non-immunologically
mediated processes, such as in the presence of bacterial toxins or
malignant hypertension, or in more chronic disease states, such as
granuloma faciale or erythema elevatum diutinum. A predominantly
lymphocytic vasculitis may represent several pathogenetic mechanisms. In

lesions more than 24 to 48 hours old, a lymphocytic vasculitis may
represent a resolving phase of an immune complex-mediated neutrophilic
vasculitis. Alternatively, this histologic pattern may be seen de novo
in
conditions with a presumed cell-mediated immunologic pathogenesis.
Lymphocytic vasculitis may also be seen in rickettsial infections such
as
Rocky Mountain spotted fever. The pathogenesis of granulomatous
vasculitis
remains poorly understood and is thought to be induced by a combination
of
circulating immune complexes and a cell-mediated immune response.

============================================================
43.) Pleural effusions in an overlap syndrome of idiopathic
hypereosinophilic syndrome and erythema elevatum diutinum.
============================================================
Eur Respir J 1990 Jan;3(1):115-8

Cordier JF, Faure M, Hermier C, Brune J
Dept. of Pneumology, Hopital Louis Pradel, Universite Claude Bernard,
Lyon,
France.

We report a patient with idiopathic hypereosinophilic syndrome
presenting
with bilateral eosinophilic pleural effusions. He also had erythema
elevatum diutinum, a rare skin disease of the vasculitic type. No
cardiomyopathy was present. Pleural effusions, skin lesions, and blood
eosinophilia disappeared with prolonged corticosteroid treatment.

============================================================
44.) Erythema elevatum diutinum: an ultrastructural case study.
============================================================
J Cutan Pathol 1989 Aug;16(4):211-7

Lee AY, Nakagawa H, Nogita T, Ishibashi Y
Department of Dermatology, Faculty of Medicine, University of Tokyo,
Japan.

An electron microscopic study was made of a patient with erythema
elevatum
diutinum, a rare variant of cutaneous leukocytoclastic vasculitis.
Biopsies
were obtained from early evolving and late fibroproliferative lesions.
Early lesions showed vasculitis and a massive dermal infiltrate composed

mainly of neutrophils, histiocytes/macrophages and apparently increased
Langerhans cells. Later lesions were characterized by a dense fibrosis
with
a proliferation of fibroblasts and myofibroblasts and a dermal
infiltrate
in which lymphocytes and histiocytes/macrophages predominated. Even in
later lesions, dermal Langerhans cells were observed in great number.
Langerhans cells may be involved in the evolution of this rare disease.

============================================================
45.) [Erythema elevatum diutinum--a rare variant of skin changes in
systemic
lupus erythematosus].
============================================================
Dermatol Monatsschr 1989;175(10):628-34

Schulze P, Audring H, Sonnichsen N
It is reported on 5 patients with erythema elevatum diutinum, in 2 of
these
cases exists a systemic lupus erythematosus and another patient suffers
from cutaneous lupus erythematosus. This information can be considered
as a
possibility that cutaneous alterations similar to erythema elevatum
diutinum could be the first symptoms of systemic lupus erythematosus
when
the usual treatment is inefficient.
===================================================================
DATA-MEDICOS/DERMAGIC-EXPRESS No 2-(84) 23/11/99 DR. JOSE LAPENTA R.
===================================================================
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MARACAY, VENEZUELA, ESTADO ARAGUA, TORRE MARACAY LAS DELICIAS P-4 OF 4-1
TELF: OFIC: 043-328571, 043-327287, CEL: 016-6401045
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Data-Médicos 
Dermagic/Express No. 2-(84) 
24 Noviembre 1.999. 24 November 1.999. 

~ Eritema Elevatum Diutinum ~ 
~ Erythema Elevatum Diutinum ~ 





EDITORIAL ESPAŃOL 
================= 
Hola amigos DERMAGICOS, el tema de hoy, EL ERITEMA ELEVATUM DIUTINUM. 
Descrito por primera vez en 1984 por Croker y William. Enfermedad poco comun clasificada dentro de las dermatosis neutrofilicas como una VASCULITIS LEUCOCITOCLASTICA ocasionada por deposision de complejos inmunes a nivel vascular. Asociada a muchas otras enfermedades DERMATOLOGICASY NO DERMATOLOGICAS como: pioderma gangrenoso, enfermedad de Crohn, sindrome mielodisplastico, granuloma facial, paraproteinemias, IgA monoclonal gammopatia, Wegener's granulomatosis, SIDA, policondritis recidivante, artritis reumatoide, lupus eritematoso sistemico, sindrome de overlap, hiperimmunoglobulinemia D sindrome, enfermedad celiaca, etc. En estas 45 refrerencias los hechos. 
En el atach eritema elevatum diutinum: palmas, codos y pies 

COMENTARIOS: 
Hay algunas peticiones de DERMAGIC que quedaron pendientes, tratare de mandarlas en este mes de vacaciones. Si el tiempo me alcanza tratare de enviar edicones cortas. Si no nos vemos de nuevo feliz ańo a todos y que el nuevo milenio nos traiga cosas mejores. 
In the attach: erythema elevatum diutinum: palm, elbow, feet 

Saludos a todos !!! 

Dr. Jose Lapenta R.,,, 

EDITORIAL ENGLISH 
================= 
Hello friends DERMAGICS, today's topic, THE ERYTHEMA ELEVATUM DIUTINUM. 
Described for the first time in 1984 by Croker and William. Not very common illness classified inside the neutrophilic diseases like a LEUKOCYTOCLASTIC VASCULITIS caused by deposision of immune complexes at vascular level. Associated to many other illnesses DERMATOLOGIC AND NON DERMATOLOGIC like: pyoderma gangrenosum, Crohn's disease, myelodisplastic syndrome, faciale granuloma, paraproteinemias, IgA monoclonal gammopathy, Wegener's granulomatosis, acquired immunodeficiency syndrome, relapsing polychondritis, rheumatoid arthritis, systemic lupus erythematosus, averlap syndrome, hyperimmunoglobulinaemia D syndrome, celiac disease etc. 
In these 45 refrerencias the facts. 

COMMENTS: 
There are some petitions of DERMAGIC that were pending, I will try to send 
them in this month of vacations. If I have enough time I will try to send 
short editions. If we don't see again happy year for all and that the new 
millennium brings us better things. !!! 


Greetings to ALL, !! 
Dr. Jose Lapenta R.,,, =================================================================== 
REFERENCIAS BIBLIOGRAFICAS / BIBLIOGRAPHICAL REFERENCES 
=================================================================== 
============================================================ 
1.) [Erythema elevatum and diutinum in Crohn disease] 
2.) Erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with intermittent plasma exchange. 
3.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases. 
4.) Terrien's marginal degeneration associated with erythema elevatum diutinum. 
5.) Erythema elevatum Diutinum. 
6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms. 
7.) Erythema elevatum diutinum: a clinicopathological study of eight cases. 
8.) Erythema elevatum diutinum with extensive acro-osteolysis. 
9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory 
reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale. 
10.) Erythema elevatum diutinum in association with coeliac disease. 
11.) Erythema elevatum diutinum complicated by rheumatoid arthritis. 
12.) Erythema elevatum diutinum in human immunodeficiency virus-infected 
patients--report of a case and review of the literature [see comments] 
13.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation. 
14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum 
diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia. 
15.) A continuum of neutrophilic disease occurring in a patient with ulcerative colitis. 
16.) Granuloma faciale with extrafacial lesions. 
17.) Erythema elevatum diutinum and HIV infection: a report of five cases. 
18.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases. 
19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory 
reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale. 
20.) [Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of associated illnesses]. 
21.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation. 
22.) Erythema elevatum diutinum in human immunodeficiency virus-infected patients--report of a case and review of the literature. 
23.) Cutaneous vasculitis. 
24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal gammopathy. 
25.) Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions. 
26.) Erythema elevatum diutinum: case report showing an unusual distribution. 
27.) Ultrastructural study of chronic lesions of erythema elevatum diutinum: "extracellular cholesterosis" is a misnomer. 
28.) Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus. 
29.) Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet. 
30.) Association of hyperimmunoglobulinaemia D syndrome with erythema elevatum diutinum. 
31.) Erythema elevatum diutinum associated with Wegener's granulomatosis and IgA paraproteinemia. 
32.) Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases. 
33.) Erythema elevatum diutinum: a clinicopathological study. 
34.) Erythema elevatum diutinum in a patient with relapsing polychondritis. 
35.) Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients. 
36.) Erythema elevatum diutinum in a patient with acquired immunodeficiency syndrome. Another clinical simulator of Kaposi's sarcoma. 
37.) Erythema elevatum diutinum--an unusual association with ulcerative colitis. 
38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda. 
39.) Cutaneous manifestations of neutrophilic disease. A study of seven cases. 
40.) Erythema elevatum diutinum--a solitary lesion in a patient with rheumatoid arthritis. 
41.) Erythema elevatum diutinum in a patient with Crohn's disease. 
42.) The natural history of vasculitis. What the histology tells us about pathogenesis. 
43.) Pleural effusions in an overlap syndrome of idiopathic hypereosinophilic syndrome and erythema elevatum diutinum. 
44.) Erythema elevatum diutinum: an ultrastructural case study. 
45.) [Erythema elevatum diutinum--a rare variant of skin changes in systemic lupus erythematosus]. 
============================================================ 
============================================================ 
1.) [Erythema elevatum and diutinum in Crohn disease] 
============================================================ 
Author 
Elsner J; Kiehl P; Kapp A; Weiss J 
Address 
Dermatologische Klinik und Poliklinik, Medizinische Hochschule Hannover. 
Source 
Hautarzt, 47(9):701-4 1996 Sep 
Abstract 
A 35-year-old woman suffering from Crohn's disease for 3 years developed 
painful erythematous nodular lesions predominantly on the dorsa of her 
hands and feet, as well as on her elbows and wrists. The clinical features 
and histopathological picture of leukocytoclastic vasculitis were both 
highly suggestive of erythema elevatum diutinum. No improvement of the skin 
lesions was observed during treatment with high doses of oral steroids. In 
contrast, treatment with dapsone resulted in a marked reduction of skin 
lesions. During a new attack of her Crohn's disease the erythema elevatum 
diutinum lesions flared again. The parallel clinical course of erythema 
elevatum diutinum and Crohn's disease, as well as the detection of immune 
complexes and neutrophilic granulocytes in lesions of both diseases, 
strongly suggest a common etiology. This hypothesis is discussed in context 
with this case and the recent literature. 
============================================================ 
2.) Erythema elevatum diutinum associated with IgA paraproteinemia successfully 
controlled with intermittent plasma exchange. 
============================================================ 
Author 
Chow RK; Benny WB; Coupe RL; Dodd WA; Ongley RC 
Address 
Division of Dermatology, University of British Columbia, Vancouver. 
Source 
Arch Dermatol, 132(11):1360-4 1996 Nov 
Abstract 
BACKGROUND: We review the literature and report a case of refractory 
erythema elevatum diutinum associated with IgA paraproteinemia that was 
successfully controlled with intermittent plasma exchange (PLEX). 
OBSERVATIONS: Typical lesions of erythema elevatum diutinum developed in a 
72-year-old patient with IgA paraproteinemia; the condition predictably 
flared whenever IgA levels reached a threshold of 8 g/L. After 8 years of 
unsuccessful treatment with various agents, we instituted a trial of PLEX 
during an acute flare. Following 6 exchanges over a period of 2 weeks, the 
IgA level decreased from 8 to 2 g/L and the skin lesions cleared. Three 
weeks later, new skin lesions developed and the IgA level had rebounded 
from 2 to just over 8 g/L. A second course of PLEX was administered, with 
excellent results, and a 3-month course of oral chlorambucil (2 mg/d) was 
initiated. The patient's condition remained in clinical remission for 10 
months. Over the ensuing 9 years, she suffered 11 further flares, each of 
which was associated with IgA levels of 8 to 10 g/L and each responding 
dramatically to 3 to 5 PLEXs followed by a consolidative dose of 
intravenous cyclophosphamide (250-500 mg). CONCLUSION: We believe that PLEX 
may have an important role in the management of severe erythema elevatum 
diutinum associated with monoclonal paraproteinemia refractory to other 

============================================================ 
3.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum and a positive rheumatoid factor: a report of three cases. 
============================================================ 
Author 
Takiwaki H; Kubo Y; Tsuda H; Arase S; Shiota H 
Address 
Department of Dermatology, School of Medicine, University of Tokushima, 
Japan. 
Source 
Br J Dermatol, 138(5):893-7 1998 May 
Abstract 
Peripheral ulcerative keratitis (PUK) is a complication of 
collagen-vascular diseases such as rheumatoid arthritis (RA) and other 
systemic vasculitides. We report three cases of erythema elevatum diutinum 
with PUK. These patients presented with nodules and plaques consistent with 
erythema elevatum diutinum on the extremities and crusted or ulcerated 
purpuric lesions on the soles. Histopathological examination of these 
lesions revealed a dense neutrophilic infiltrate with nuclear dust and 
fibrin around blood vessels. All the patients developed PUK concomitant 
with the development of the skin lesions. The rheumatoid factor was 
positive at high titre in all three patients; this was associated with 
probable RA in one. Cutaneous lesions were dramatically improved by 
administration of dapsone in all cases. Dapsone was also effective in 
treating the ocular lesions in two patients. 

============================================================ 
4.) Terrien's marginal degeneration associated with erythema elevatum diutinum. 
============================================================ 
Author 
Shimazaki J; Yang HY; Shimmura S; Tsubota K 
Address 
Department of Ophthalmology, Tokyo Dental College, Chiba, Japan. 
Source 
Cornea, 17(3):342-4 1998 May 
Abstract 
PURPOSE: To describe a patient with Terrien's marginal degeneration 
associated with a chronic skin disease, erythema elevatum diutinum. 
METHODS: A 27-year-old woman with peripheral thinning of her left cornea 
associated with adjacent conjunctival and episcleral inflammation was 
clinically examined. RESULTS: Episodes of worsening skin lesions were 
associated with development of inflammation in her left eye. Administration 
of sulfones was effective for reducing both skin and ocular inflammation. 
CONCLUSION: Erythema elevatum diutinum should be considered an underlying 
systemic disease of Terrien's marginal degeneration. 
============================================================ 
5.) Erythema elevatum diutinum. 
============================================================ 
Author: 
Croker H W, Williams C. 
Br J Dermatol 1984;6:1-9,33,8 
============================================================ 
============================================================ 
6.) Nodular erythema elevatum diutinum mimicking cutaneous neoplasms. 
Author 
============================================================ 
Shanks JH; Banerjee SS; Bishop PW; Pearson JM; Eyden BP 
Address 
Department of Histopathology, Christie Hospital NHS Trust, UK. 
Source 
Histopathology, 31(1):91-6 1997 Jul 
Abstract 
AIMS: We describe the cutaneous pseudoneoplastic lesions in two patients 
with nodular erythema elevatum diutinum, a rare chronic disorder in which 
polymorph nuclear fragmentation (leukocytoclasis) is present within dermal 
nodules showing spindle cells and fibrosis. In both cases diagnostic 
difficulty was encountered clinically and pathologically and various benign 
and malignant neoplasms were considered in the differential diagnosis. 
METHODS AND RESULTS: Immunohistochemically the spindle cells were negative 
for CAM5.2, AE1/3, S100 protein and desmin (D33). They were positive for 
vimentin and focally positive for CD34 and alpha-smooth muscle actin. Some 
of the spindle cells were positive for Mac 387 and KP1(CD68). By electron 
microscopy, the lesions were shown to consist of fibroblasts/myofibroblasts 
and fusiform macrophages. CONCLUSIONS: Increased awareness of the features 
described will help to avoid misdiagnosis as a neoplastic process. 

============================================================ 
7.) Erythema elevatum diutinum: a clinicopathological study of eight cases. 
Author 
============================================================ 
Sang¨ueza OP; Pilcher B; Martin Sang¨ueza J 
Address 
Department of Pathology, Medical College of Georgia, Augusta 30912, USA. 
Source 
Am J Dermatopathol, 19(3):214-22 1997 Jun 
Abstract 
Erythema elevatum diutinum (EED) is a rare cutaneous condition that 
initially presents as leukocytoclastic vasculitis (LCCV) of the skin and 
later resolves with fibrosis. In addition to the LCCV, EED may show 
features reminiscent of other entities. For example, it may mimic lesions 
of dermatofibroma, granuloma annulare, granuloma faciale, or dermatitis 
herpetiformis. For this study, we reviewed the clinical records and 13 skin 
biopsies in eight patients with EED. One of the patients had concurrent 
pityriasis rubra pilaris, and another developed lesions of EED following 
and at the sites of mosquito bites; these associations have not been noted 
previously. In addition to such typical histopathological features as 
diffuse dermal involvement by neutrophils, eosinophils, and 
leukocytoclastic vasculitis, we also found two unusual patterns. The first 
was characterized by palisaded necrotizing granulomas, as previously 
described and associated with Churg-Strauss granuloma; the second condition 
simulated a pyogenicgranuloma--like lesion. 

============================================================ 
8.) Erythema elevatum diutinum with extensive acro-osteolysis. 
Author 
============================================================ 
Ellabban A; Schumacher HR Jr 
Address 
Division of Rheumatology, University of Pennsylvania School of Medicine, 
Philadelphia, USA. 
Source 
J Rheumatol, 24(6):1203-5 1997 Jun 
Abstract 
Erythema elevatum diutinum (EED) is a rare chronic skin disease with 
cutaneous vasculitis, characterized by the absence of systemic 
vasculopathy. We describe a 79-year-old white woman who has been followed 
with the diagnosis of EED for 27 years. Our patient has a previously 
unreported combination of EED and acro-osteolysis. After reviewing possible 
reasons for the association, her vasculitis seems the most likely factor. 

============================================================ 
9.) Localized chronic fibrosing vasculitis of the skin: an inflammatory 
reaction that occurs in settings other than erythema elevatum diutinum and 
granuloma faciale. 
============================================================ 
Author 
Carlson JA; LeBoit PE 
Address 
Department of Pathology, Albany Medical College, New York, USA. 
Source 
Am J Surg Pathol, 21(6):698-705 1997 Jun 
Abstract 
Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, 
localized forms of cutaneous leukocytoclastic vasculitis that result in 
patterned (storiform or concentric) fibrosis. EED often occurs in 
systemically ill patients as bilaterally symmetrical plaques, papules, or 
nodules, often over the dorsa of joints. GF occurs as one or a few plaques 
on the face. Eosinophils and plasma cells are prominent in GF, whereas 
neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany 
facial ones in GF, and there are a few reports of upper respiratory tract 
masses with GF-like histologic features. We report on eight patients with 
solitary cutaneous lesions with histologic features similar to those of EED 
or GF, but whose clinical picture was not that of either disease. One, 
whose histology resembled GF, had a large multinodular dermal and 
subcutaneous mass that persisted despite attempted resection. Unusual 
histologic findings in other cases included storiform fibrosis with dense 
infiltrates of plasma cells, branching nerve fascicles admixed with 
EED-like changes, and EED-like areas adjacent to zones mimicking a 
sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the 
stereotypic settings of GF and EED and is an inflammatory reaction pattern 
that does not signify a specific diagnosis. Because of transitions between 
EED or GF-like areas and those of patterned sclerosis with plasma cell-rich 
infiltrates, we believe that some "inflammatory pseudotumors" of the skin, 
and perhaps of other sites could be the result of localized vasculitis. 

============================================================ 
10.) Erythema elevatum diutinum in association with coeliac disease. 
Author 
============================================================ 
Tasanen K; Raudasoja R; Kallioinen M; Ranki A 
Address 
Department of Dermatology, Oulu University Hospital, Finland. 
Source 
Br J Dermatol, 136(4):624-7 1997 Apr 
Abstract 
Erythema elevatum diutinum (EED) has been described in association with 
several immunological or infectious diseases. We describe a female patient 
who presented with clinically and histologically typical EED in whom 
previously undiagnosed coeliac disease was found. Appearance of EED lesions 
was preceded by widespread joint pains. In extensive laboratory tests, the 
only abnormal findings were an elevated erythrocyte sedimentation rate 
(ESR) and decreased haemoglobin and folic acid levels. Later, IgA and IgG 
type antireticulin and antigliadin antibodies were detected. Serum total 
IgA was elevated but no paraproteinaemia was found. In lesional skin, 
granular deposits of IgA and C3 were seen at the dermo-epidermal junction. 
A duodenal biopsy revealed total villous atrophy. Dapsone treatment was 
partly effective but complete healing of the EED lesions was achieved only 
after the introduction of a strict gluten-free diet. The patient has now 
remained symptom-free on the diet for 1.5 years. 

============================================================ 
11.) Erythema elevatum diutinum complicated by rheumatoid arthritis. 
============================================================ 
Author 
Nakajima H; Ikeda M; Yamamoto Y; Kodama H 
Address 
Department of Dermatology, Kochi Medical School, Japan. 
Source 
J Dermatol, 26(7):452-6 1999 Jul 
Abstract 
A 53-year-old female developed erythema elevatum diutinum (EED) twelve 
years after the onset of rheumatoid arthritis. The arthritis had been well 
controlled for the last several years. Annular purpuric macules were 
characteristically complicated by common nodular and plaque lesions. Both 
leukocytoclastic vasculitis and fibrosis were observed in the macular 
lesions, indicating that the lesions were a manifestation of an early phase 
of EED. Both types of skin lesions disappeared with treatment with dapsone. 
They have not relapsed for two years after stopping the dapsone. The 
leukocytoclastic vasculitis was thought to have developed independently of 
the rheumatoid arthritis. She had noticed sicca symptoms two years before 
the appearance of EED, but she did not satisfy the diagnostic criteria for 
Sj¨ogren's syndrome. 

============================================================ 
12.) Erythema elevatum diutinum in human immunodeficiency virus-infected 
patients--report of a case and review of the literature [see comments] 
============================================================ 
Author 
Dronda F; Gonz´alez-L´opez A; Lecona M; Barros C 
Address 
Hospital General Penitenciario, Madrid, Spain. 
Source 
Clin Exp Dermatol, 21(3):222-5 1996 May 
Abstract 
Erythema elevatum diutinum (EED) is a rare chronic disease of unknown 
origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A 
case of EED in a 32-year-old HIV-infected male patient, with no previous 
opportunistic infections and a CD4+ cell count of less than 200/mm3, is 
reported. Therapy with oral dapsone (100 mg/day for 15 days) resulted in 
clinical cure with no relapse after 6 months of follow-up. To our 
knowledge, only six cases of EED in HIV-positive patients have been 
reported to date. A brief review of these seven cases is described. 

============================================================ 
13.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation. 
============================================================ 
Author 
Porneuf M; Duterque M; Sotto A; Jourdan J 
Address 
Department of Internal Medicine, University of Nimes, France. 
Source 
Br J Dermatol, 134(6):1131-4 1996 Jun 
Abstract 
A 78-year-old woman with a history of symmetrical erythematous plaques on 
the arms, and a monoclonal gammopathy, developed a strange striped 
reticulate papular dermatosis with central atrophy. Histological 
examination was compatible with a very late stage of erythema elevatum 
diutinum (EED), showing a fibrohistiocytic proliferation with areas of 
granulation tissue. This fibrosis may result from the chronic dermal injury 
of leucocytoclastic vasculitis and is sometimes the predominant histology 
of EED. Investigations for underlying haematological anomalies, such as 
paraproteinaemia and infection with human immunodeficiency virus, must be 
performed. Dapsone is ineffective once the fibrous nodules have appeared. 

============================================================ 
14.) POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum 
diutinum, and rheumatoid arthritis as extramedullary manifestations of 
plasma cell dyscrasia. 
============================================================ 
Author 
Albitar S; Bourgeon B; Genin R; Jacquesson M; Riviere JP; Serveaux MO; 
Ribera A; Serveaux JP 
Address 
Hemodialysis Center, AURAR, 6 Avenue Stanislas Gimart, F-97490 Ste Clotilde 
de La Reunion, France. 
Source 
Am J Kidney Dis, 31(4):E3 1998 Apr 
Abstract 
POEMS syndrome is a rare synopsis of different multisystemic disorders 
(polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and 
skin lesions) associated with plasma cell dyscrasia. We herein report the 
atypical case of a 44-year-old white man presenting with glomerulopathy, 
POEMS syndrome, and erythema elevatum diutinum with a few-year history of 
non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid 
arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The 
prescription of 1 mg/kg/day prednisone improved the patient's features 
dramatically. Skin lesions improved by the association of glucocorticoids 
and plasma exchange, recurred when plasmapheresis ceased, and remitted when 
plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred 
when corticoids were discontinued and remitted when prednisone was 
reintroduced. However, prednisone and plasmapheresis had no effect on 
polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, 
who developed indolent multiple myeloma a few years later. We thus 
concluded that POEMS syndrome, steroid-dependent diabetes mellitus, 
rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered 
by plasma cell dyscrasia. 

============================================================ 
15.) A continuum of neutrophilic disease occurring in a patient with ulcerative 
colitis. 
============================================================ 
Author 
Salmon P; Rademaker M; Edwards L 
Address 
Department of Dermatology, Waikato Hospital, Health Waikato, Hamilton, New 
Zealand. 
Source 
Australas J Dermatol, 39(2):116-8 1998 May 
Abstract 
A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) 
occurring concurrently with bullous pyoderma gangrenosum is reported to 
emphasize the close relationship between these two disorders. Atypical 
pyoderma gangrenosum and Sweet's syndrome have been described as occurring 
simultaneously in haematological dyscrasias but not, to our knowledge, in 
ulcerative colitis. It has been proposed that pyoderma gangrenosum, Sweet's 
syndrome, erythema elevatum diutinum and subcorneal pustular dermatosis may 
represent manifestations along a continuum of neutrophilic dermatoses. 

============================================================ 
16.) Granuloma faciale with extrafacial lesions. 
============================================================ 
Author 
Roustan G; S´anchez Yus E; Salas C; Sim´on A 
Address 
Department of Dermatology, Cl´inica Universitaria Puerta de Hierro, Madrid, 
Espa~na. 
Source 
Dermatology, 198(1):79-82 1999 
Abstract 
Extrafacial involvement in granuloma faciale (GF) is rather exceptional. We 
report herein a patient with GF associated with lesions on the trunk and 
the forearm. Histological studies of facial and extrafacial lesions shared 
similar characteristics: a mixed inflammatory infiltrate with abundant 
eosinophils in the superficial and middle dermis with a narrow grenz zone 
of uninvolved dermis between the epidermis and the infiltrate. Evidence of 
vasculitis was clearly observed in both biopsy specimens. Treatment with 
dapsone did not alter the course of the disease. We review the 12 cases of 
extrafacial GF that have been reported in the English and Spanish 
literature. In these cases a diagnosis of erythema elevatum diutinum (EED) 
may be suggested. Although GF and EED may share some pathogenic mechanisms, 
there are several clinical and histological differences between them that 
make us consider EED and GF as distinct entities. 

============================================================ 
17.) Erythema elevatum diutinum and HIV infection: a report of five cases. 
============================================================ 
Br J Dermatol 1999 Aug;141(2):335-338 

Muratori S, Carrera C, Gorani A, Alessi E 
Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale 
Maggiore, Via Pace 9, 20122 Milan, Italy. 


Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated 
dermatosis, 11 cases having so far been reported in the medical literature 
and five patients with the disease having been seen by us during the last 4 
years. As the disease is poorly known, it is easily confused with Kaposi's 
sarcoma or bacillary angiomatosis, but the histopathological features are 
diagnostic. EED is considered to be an immune complex-mediated vasculitis. 
A streptococcal infection seemed to be the trigger factor in four of our 
patients. Partial control of the cutaneous lesions was achieved by the use 
of antibiotics. 

============================================================ 
18.) Peripheral ulcerative keratitis associated with erythema elevatum diutinum 
and a positive rheumatoid factor: a report of three cases. 
============================================================ 
Br J Dermatol 1998 May;138(5):893-7 

Takiwaki H, Kubo Y, Tsuda H, Arase S, Shiota H 
Department of Dermatology, School of Medicine, University of Tokushima, 
Japan. 

Peripheral ulcerative keratitis (PUK) is a complication of 
collagen-vascular diseases such as rheumatoid arthritis (RA) and other 
systemic vasculitides. We report three cases of erythema elevatum diutinum 
with PUK. These patients presented with nodules and plaques consistent with 
erythema elevatum diutinum on the extremities and crusted or ulcerated 
purpuric lesions on the soles. Histopathological examination of these 
lesions revealed a dense neutrophilic infiltrate with nuclear dust and 
fibrin around blood vessels. All the patients developed PUK concomitant 
with the development of the skin lesions. The rheumatoid factor was 
positive at high titre in all three patients; this was associated with 
probable RA in one. Cutaneous lesions were dramatically improved by 
administration of dapsone in all cases. Dapsone was also effective in 
treating the ocular lesions in two patients. 

============================================================ 
19.) Localized chronic fibrosing vasculitis of the skin: an inflammatory 
reaction that occurs in settings other than erythema elevatum diutinum and 
granuloma faciale. 
============================================================ 
Am J Surg Pathol 1997 Jun;21(6):698-705 

Carlson JA, LeBoit PE 
Department of Pathology, Albany Medical College, New York, USA. 

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, 
localized forms of cutaneous leukocytoclastic vasculitis that result in 
patterned (storiform or concentric) fibrosis. EED often occurs in 
systemically ill patients as bilaterally symmetrical plaques, papules, or 
nodules, often over the dorsa of joints. GF occurs as one or a few plaques 
on the face. Eosinophils and plasma cells are prominent in GF, whereas 
neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany 
facial ones in GF, and there are a few reports of upper respiratory tract 
masses with GF-like histologic features. We report on eight patients with 
solitary cutaneous lesions with histologic features similar to those of EED 
or GF, but whose clinical picture was not that of either disease. One, 
whose histology resembled GF, had a large multinodular dermal and 
subcutaneous mass that persisted despite attempted resection. Unusual 
histologic findings in other cases included storiform fibrosis with dense 
infiltrates of plasma cells, branching nerve fascicles admixed with 
EED-like changes, and EED-like areas adjacent to zones mimicking a 
sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the 
stereotypic settings of GF and EED and is an inflammatory reaction pattern 
that does not signify a specific diagnosis. Because of transitions between 
EED or GF-like areas and those of patterned sclerosis with plasma cell-rich 
infiltrates, we believe that some "inflammatory pseudotumors" of the skin, 
and perhaps of other sites could be the result of localized vasculitis. 

============================================================ 
20.) [Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of 
associated illnesses]. 
============================================================ 
Hautarzt 1997 Feb;48(2):113-7 

Gerbig AW, Zala L, Hunziker T 
Dermatologische Universitatsklinik, Bern. 

Erythema elevatum diutinum (EED) is a rare disease presenting with 
persistent red to yellow-brown papules or plaques which are mainly 
localized symmetrically on the extensor aspects of the hands and fingers, 
the elbows and the knees. The histology shows a leucocytoclastic vasculitis 
in early lesions and fibrosis of the dermis later on. Dapsone is the 
treatment of choice. Today, EED is usually assigned to the neutrophilic 
dermatoses in which an association with hematological disturbances is well 
documented. We report on a patient with EED and glioma WHO grade IV, a 
coincidence unreported hitherto. Additionally, we review the literature on 
diseases associated with EED. 

============================================================ 
21.) Unusual erythema elevatum diutinum with fibrohistiocytic proliferation. 
============================================================ 
Br J Dermatol 1996 Jun;134(6):1131-4 

Porneuf M, Duterque M, Sotto A, Jourdan J 
Department of Internal Medicine, University of Nimes, France. 

A 78-year-old woman with a history of symmetrical erythematous plaques on 
the arms, and a monoclonal gammopathy, developed a strange striped 
reticulate papular dermatosis with central atrophy. Histological 
examination was compatible with a very late stage of erythema elevatum 
diutinum (EED), showing a fibrohistiocytic proliferation with areas of 
granulation tissue. This fibrosis may result from the chronic dermal injury 
of leucocytoclastic vasculitis and is sometimes the predominant histology 
of EED. Investigations for underlying haematological anomalies, such as 
paraproteinaemia and infection with human immunodeficiency virus, must be 
performed. Dapsone is ineffective once the fibrous nodules have appeared. 

============================================================ 
22.) Erythema elevatum diutinum in human immunodeficiency virus-infected 
patients--report of a case and review of the literature. 
============================================================ 
Clin Exp Dermatol 1996 May;21(3):222-5 

Dronda F, Gonzalez-Lopez A, Lecona M, Barros C 
Hospital General Penitenciario, Madrid, Spain. 

Erythema elevatum diutinum (EED) is a rare chronic disease of unknown 
origin, part of the spectrum of cutaneous leucocytoclastic vasculitis. A 
case of EED in a 32-year-old HIV-infected male patient, with no previous 
opportunistic infections and a CD4+ cell count of less than 200/mm3, is 
reported. Therapy with oral dapsone (100 mg/day for 15 days) resulted in 
clinical cure with no relapse after 6 months of follow-up. To our 
knowledge, only six cases of EED in HIV-positive patients have been 
reported to date. A brief review of these seven cases is described. 

============================================================ 
23.) Cutaneous vasculitis. 
============================================================ 
Rheum Dis Clin North Am 1995 Nov;21(4):1097-113 

Gibson LE, Su WP 
Mayo Clinic, Rochester, Minnesota, USA. 

Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and 
histopathologic findings that share the common theme of vascular 
inflammation and blood vessel damage. Clinical pattern and proper 
histopathologic studies are essential to the proper classification and 
evaluation of vasculitis. This article discusses the major types of 
cutaneous vasculitis typified by necrotizing or leukocytoclastic vasculitis 
and includes discussions of Henoch-Schonlein purpura, urticarial 
vasculitis, erythema elevatum diutinum, cutaneous polyarteritis nodosa, 
livedoid vasculitis, and cutaneous granulomatous vasculitis. 

============================================================ 
24.) Pyoderma gangrenosum, erythema elevatum diutinum and IgA monoclonal 
gammopathy. 
============================================================ 
Australas J Dermatol 1995 Feb;36(1):21-3 

Wayte JA, Rogers S, Powell FC 
Regional Centre of Dermatology, Mater Misericordiae Hospital, Dublin, 
Ireland. 

A patient with erythema elevatum diutinum (EED) developed pyoderma 
gangrenosum (PG). Investigation revealed an IgA kappa monoclonal 
gammopathy. Previous reports of PG in association with EED are reviewed and 
the spectrum of the neutrophilic dermatoses discussed. 

============================================================ 
25.) Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions. 
============================================================ 
Br J Dermatol 1994 Jul;131(1):112-7 

Aractingi S, Bachmeyer C, Dombret H, Vignon-Pennamen D, Degos L, Dubertret L 
Department of Dermatology, H pital Saint-Louis, Paris, France. 

We report the concomitant occurrence of erythema elevatum diutinum and 
specific skin lesions in a patient with a myelodysplastic syndrome (MDS). 
This patient's course, and review of other reported cases, support the 
opinion that neutrophilic dermatoses are associated with a poor prognosis 
of MDS. The simultaneous appearance of these manifestations could be the 
consequence of a particular chemotactism of myeloid cells, expressed after 
acute transformation. 

============================================================ 
26.) Erythema elevatum diutinum: case report showing an unusual distribution. 
============================================================ 
Cutis 1994 Mar;53(3):124-6 

Hansen U, Haerslev T, Knudsen B, Jacobsen GK 
Department of Pathology, Gentofte Hospital, University of Copenhagen, 
Denmark. 

A case of erythema elevatum diutinum showing an unusual distribution is 
reported. Hard nodules were located on the patient's fingertips and pulps 
while the extensor surfaces were not involved. The disease was initially 
misinterpreted based on both clinical and histologic examination. Dapsone 
therapy was followed by a remarkable recovery. The clinical and histologic 
features of erythema elevatum diutinum are summarized. 

============================================================ 
27.) Ultrastructural study of chronic lesions of erythema elevatum diutinum: 
"extracellular cholesterosis" is a misnomer. 
============================================================ 
J Am Acad Dermatol 1993 Aug;29(2 Pt 2):363-7 

Kanitakis J, Cozzani E, Lyonnet S, Thivolet J 
Department of Dermatology, Ed. Herriot Hospital, Lyon, France. 

Erythema elevatum diutinum (EED) is a rare disease of unknown origin that 
belongs to the spectrum of leukocytoclastic vasculitis. Chronic lesions of 
EED contain lipid deposits, for which the term extracellular cholesterosis 
has been coined. We studied a typical case of EED with long-standing 
lesions. Findings of electron microscopic examination revealed a heavy, 
exclusively intracellular lipid deposition that consisted of lipid 
droplets, myelin figures, and rare cholesterol clefts within histiocytes 
but also within epidermal keratinocytes, mast cells, pericytes, and 
lymphocytes. These findings are in keeping with the results of previous 
ultrastructural studies and suggest that the term extracellular 
cholesterosis is a misnomer; intracellular lipidosis would more accurately 
describe the lipid deposition. 

============================================================ 
28.) Nodular lesions of erythema elevatum diutinum in patients infected with the human immunodeficiency virus. 
============================================================ 
J Am Acad Dermatol 1993 Jun;28(6):919-22 

LeBoit PE, Cockerell CJ 
Department of Pathology, School of Medicine, University of California, San 
Francisco 94143-0506. 

BACKGROUND: Erythema elevatum diutinum (EED) is a rare, chronic form of 
cutaneous vasculitis that can result in fibrosis. Four patients infected 
with the human immunodeficiency virus (HIV) had unusual nodular lesions of 
EED. OBJECTIVE: Our purpose was to document the occurrence of these lesions 
in HIV-infected patients and to characterize the histopathologic and 
immunophenotypic features of nodular EED. METHODS: Clinicopathologic 
studies were supplemented by special stains of biopsy specimens. RESULTS: 
EED may be a complication of HIV infection. Nodules of EED are composed of 
small aggregates of Mac-387+ spindled cells. Within the aggregates can be 
seen neutrophils, nuclear dust, and fibrin. The marked fibrosis of the 
lesions may account for the lack of response to dapsone. CONCLUSION: EED 
should be considered in the differential diagnosis of juxta-articular 
nodules in HIV-infected patients; its diagnosis can be confirmed by biopsy. 

============================================================ 
29.) Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet. 
============================================================ 
Pediatr Dermatol 1993 Jun;10(2):125-8 

Rodriguez-Serna M, Fortea JM, Perez A, Febrer I, Ribes C, Aliaga A 
Department of Dermatology, Hospital General, Valencia, Spain. 

An 11-year-old girl with a history of insulin-dependent diabetes mellitus 
had erythema elevatum diutinum (EED) associated with a celiac disease 
related to a possible kidney disease. Dapsone did not improve the skin 
manifestations. However, the lesions disappeared after a gluten-free diet 
was begun. To our knowledge, this report describes the first case of EED in 
a patient with celiac disease. 

============================================================ 
30.) Association of hyperimmunoglobulinaemia D syndrome with erythema elevatum diutinum. 
============================================================ 
Br J Dermatol 1993 May;128(5):572-4 

Miyagawa S, Kitamura W, Morita K, Saishin M, Shirai T 
Department of Dermatology, Nara Medical University, Japan. 

Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a 
female patient with erythema elevatum diutinum. The association of this 
skin disease with hyperimmunoglobulinaemia D may indicate a pathogenetic 
relationship. The skin lesions responded to dapsone therapy. 

============================================================ 
31.) Erythema elevatum diutinum associated with Wegener's granulomatosis and IgA paraproteinemia. 
============================================================ 
J Am Acad Dermatol 1993 May;28(5 Pt 2):846-9 

Kavanagh GM, Colaco CB, Bradfield JW, Archer CB 
Department of Dermatology, Bristol Royal Infirmary, University of Bristol, 
United Kingdom. 

A 69-year-old man had erythema elevatum diutinum for several years before 
he developed IgA paraproteinemia and a limited form of Wegener's 
granulomatosis. This is the first report of an association between erythema 
elevatum diutinum and Wegener's granulomatosis. IgA paraproteinemia has 
been reported in association with erythema elevatum diutinum but not with 
Wegener's granulomatosis. 

============================================================ 
32.) Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases. 
============================================================ 
Cutis 1992 Mar;49(3):261-6 

Planaguma M, Puig L, Alomar A, Matias-Guiu X, de Moragas JM 
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Autonomous 
University of Barcelona, Spain. 

We report on the appearance of centrifugally spreading ulcers with 
undermined borders in two patients with chronic recurrent erythema elevatum 
diutinum controlled with dapsone. The ulcerated lesions were consistent on 
clinical and pathologic examination with the diagnosis of pyoderma 
gangrenosum. They eventually responded to treatment with oral 
corticosteroids. The addition of cyclosporine was required in one case. No 
associated disease was found in any of the patients. The possible 
pathophysiological mechanisms of this uncommon association are reviewed. 

============================================================ 
33.) Erythema elevatum diutinum: a clinicopathological study. 
============================================================ 
Clin Exp Dermatol 1992 Mar;17(2):87-93 

Wilkinson SM, English JS, Smith NP, Wilson-Jones E, Winkelmann RK 
Department of Dermatology, North Staffordshire Hospital Centre, 
Stoke-on-Trent, U.K. 

Erythema elevatum diutinum is a syndrome of vasculitis in which lesions, 
typically over the extensor surfaces, showed a mixed inflammatory 
infiltrate on biopsy. We describe a series of 13 patients. The most common 
association in our series was with hypergammaglobulinemia; both mono and 
polyclonal. Chronic infection, not streptococcal, was a less frequent 
finding although two of three patients had a positive reaction to the 
intradermal injection of streptococcal antigen. Dapsone remains the initial 
treatment of choice. 

============================================================ 
34.) Erythema elevatum diutinum in a patient with relapsing polychondritis. 
============================================================ 
J Am Acad Dermatol 1992 Feb;26(2 Pt 2):312-5 

Bernard P, Bedane C, Delrous JL, Catanzano G, Bonnetblanc JM 
Department of Dermatology, CHRU Dupuytren, Limoges, France. 

A 69-year-old man with a history of relapsing polychondritis developed a 
widespread cutaneous eruption that was clinically and histologically 
typical of erythema elevatum diutinum. This eruption was accompanied by a 
recurrence of arthralgias. The significance of this association between 
relapsing polychondritis and erythema elevatum diutinum, which has not been 
previously reported, is discussed. 

============================================================ 
35.) Erythema elevatum diutinum: a clinical and histopathologic study of 13 
patients. 
============================================================ 
J Am Acad Dermatol 1992 Jan;26(1):38-44 

Yiannias JA, el-Azhary RA, Gibson LE 
Department of Dermatology, Mayo Clinic, Rochester, MN 55905. 

BACKGROUND: Erythema elevatum diutinum is a rare condition representing a 
chronic leukocytoclastic vasculitis. OBJECTIVE: Clinical and laboratory 
features of the disease were reviewed to better understand the disease. 
METHODS: The medical records and histopathologic slides of 13 patients with 
erythema elevatum diutinum were studied. RESULTS: The lesions were 
violaceous, deep red, or brown and typically were papules or plaques. 
Lesions were most often located on the extensor surfaces of the 
extremities. Associated medical problems included hematologic abnormalities 
in six patients: IgA clonal gammopathies (four), multiple myeloma (one), 
and myelodysplasia (one). Erythema elevatum diutinum preceded the 
myeloproliferative disorders by an average of 7.8 years. All patients 
showed vasculitis. Leukocytoclasia was present in 27 of 35 specimens. The 
predominant cell type in the inflammatory infiltrate was polymorphonuclear 
leukocytes or a mixture of polymorphonuclear leukocytes and lymphocytes. 
CONCLUSION: The most significant finding of this study is the association 
of erythema elevatum diutinum with hematologic disease, most frequently an 
IgA monoclonal gammopathy. 

============================================================ 
36.) Erythema elevatum diutinum in a patient with acquired immunodeficiency 
syndrome. Another clinical simulator of Kaposi's sarcoma. 
============================================================ 
Arch Dermatol 1991 Dec;127(12):1819-22 

Requena L, Sanchez Yus E, Martin L, Barat A, Arias D 
Department of Dermatology, Jimenez Diaz Foundation, Nuestra Senora de la 
Concepcion Clinic, Universidad Autonoma, Madrid, Spain. 

Several types of vasculitis have been described in patients with human 
immunodeficiency virus infection. Erythema elevatum diutinum is a rare 
variant of cutaneous leukocytoclastic vasculitis which, with the exception 
of the case reported herein, has been described only once in human 
immunodeficiency virus-infected patients. Our male patient, a longtime 
intravenous drug abuser, had cutaneous lesions, closely resembling Kaposi's 
sarcoma, on the extensor surfaces of the lower extremities. Cutaneous 
biopsy specimens, however, demonstrated leukocytoclastic vasculitis with 
fibrinoid necrosis of the vessel walls and areas of basophilic degeneration 
of collagen bundles in early lesions, whereas late lesions showed dense 
diffuse fibrosis with proliferation of dermal spindle cells and some foci 
of residual leukocytoclastic vasculitis. Oral therapy with dapsone resulted 
in marked clearing of the cutaneous lesions within few days. This case 
raises the necessity of histologic confirmation for all cases of suspected 
Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. We 
discuss the possible pathogenesis of leukocytoclastic vasculitis in human 
immunodeficiency virus-infected patients. 

============================================================ 
37.) Erythema elevatum diutinum--an unusual association with ulcerative colitis. 
============================================================ 
Clin Exp Dermatol 1991 May;16(3):204-6 

Buahene K, Hudson M, Mowat A, Smart L, Ormerod AD 
Department of Dermatology, Aberdeen Royal Infirmary, UK. 

We report the association of erythema elevatum diutinum (EED) with 
ulcerative colitis (UC). The patient, whose history included rheumatoid 
disease, diabetes mellitus (type I) and hypothyroidism developed EED during 
a severe acute exacerbation of UC which resolved following colectomy. To 
our knowledge this is the first report of such an association. Also of 
interest was the observation of Koebner phenomenon occurring at the sites 
where blood vessels were damaged. 

============================================================ 
38.) Erythema elevatum diutinum mimicking porphyria cutanea tarda. 
============================================================ 
Br J Dermatol 1991 Jan;124(1):89-91 

Requena L, Barat A, Hasson A, Arias D, Gutierrez MC, Martin L, De Castro A 
Department of Dermatology, Universidad Autonoma, Madrid, Spain. 

A case of erythema elevatum diutinum (EED) closely resembling porphyria 
cutanea tarda (PCT) is reported. The initial skin biopsies were suggestive 
for PCT but porphyrin levels in the urine, stool and plasma were normal. A 
further biopsy from an early cutaneous lesion showed a leucocytoclastic 
vasculitis with fibrinoid necrosis of the vessel walls. 

============================================================ 
39.) Cutaneous manifestations of neutrophilic disease. A study of seven cases. 
============================================================ 
Dermatologica 1991;183(4):255-64 

Vignon-Pennamen MD, Wallach D 
Department of Dermatology, Hopital Saint-Louis, Paris, France. 

Seven patients with a complex form of neutrophilic dermatosis are reported. 
Clinically, they had variable associations of four types of lesions: 
blisters/pustules, plaques, nodules and ulcerations. Histologically, a 
neutrophilic infiltrate was observed at variable levels in the epidermis, 
dermis and subcutis. Systemic manifestations were present in all cases 
(general symptoms, joint, renal, ocular and lung involvements). Three 
patients had an associated disease (myelodysplasia, metastatic carcinoma, 
IgG gammopathy). Steroids were the most efficient treatment. These 
observations, as well as a review of the literature, support the opinion 
that the neutrophilic dermatosis represents a continuous spectrum 
encompassing four well-defined entities: subcorneal pustular dermatosis, 
Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We 
propose that the different patterns of the neutrophilic dermatosis are the 
most obvious manifestations of a potentially multisystemic neutrophilic 
disease and allow its recognition. 

============================================================ 
40.) Erythema elevatum diutinum--a solitary lesion in a patient with rheumatoid arthritis. 
============================================================ 
Clin Exp Dermatol 1990 Sep;15(5):394-5 

Collier PM, Neill SM, Branfoot AC, Staughton RC 
Department of Dermatology, Westminster Hospital, London, UK. 

Erythema elevatum diutinum is a rare disease of unknown aetiology. It is 
usually symmetrical with multiple lesions. An association with rheumatoid 
has previously been reported. We report a case of atypical erythema 
elevatum diutinum affecting the right elbow of a female patient with 
seropositive rheumatoid arthritis. 

============================================================ 
41.) Erythema elevatum diutinum in a patient with Crohn's disease. 
============================================================ 
J Am Acad Dermatol 1990 May;22(5 Pt 2):948-52 

Walker KD, Badame AJ 
Department of Dermatology, Tulane University Medical School, New Orleans, 
LA 70112. 

A possible association between erythema elevatum diutinum and Crohn's 
disease is described. To our knowledge, erythema elevation diutinum has 
never been reported in a patient with Crohn's disease, nor has the disorder 
been considered one of the cutaneous manifestations of inflammatory bowel 
disease. Erythema elevatum diutinum is believed to be mediated by the 
deposition of circulating immune complexes in the dermal perivascular 
spaces. Our patient showed clinical and histopathologic findings typical of 
both erythema elevatum diutinum and Crohn's disease. The patient also had a 
positive Raji cell assay, which demonstrates the presence of a circulating 
immune complex. Increased levels of circulating immune complexes have been 
found in patients with inflammatory bowel disease, and the gastrointestinal 
inflammation in Crohn's disease could lead to enhanced antigenic exposure 
and increased formation of immune complexes. We contend that inflammatory 
bowel disease may have contributed to the development of erythema elevatum 
diutinum in our patient. 

============================================================ 
42.) The natural history of vasculitis. What the histology tells us about 
pathogenesis. 
============================================================ 
Arch Dermatol 1990 Jan;126(1):84-9 

Smoller BR, McNutt NS, Contreras F 
Department of Medicine (Dermatology), New York Hospital-Cornell University 
Medical Center, NY 10021. 

While histopathologic analysis may offer some clues as to the pathogenesis 
of vasculitis, observations must be interpreted with caution, as there is 
considerable overlap in the histologic pattern. In most cases, a 
predominantly neutrophilic vasculitis affecting small dermal venules 
suggests a relatively acute, immune complex-mediated reaction. Less 
commonly, this histologic pattern may be seen in non-immunologically 
mediated processes, such as in the presence of bacterial toxins or 
malignant hypertension, or in more chronic disease states, such as 
granuloma faciale or erythema elevatum diutinum. A predominantly 
lymphocytic vasculitis may represent several pathogenetic mechanisms. In 
lesions more than 24 to 48 hours old, a lymphocytic vasculitis may 
represent a resolving phase of an immune complex-mediated neutrophilic 
vasculitis. Alternatively, this histologic pattern may be seen de novo in 
conditions with a presumed cell-mediated immunologic pathogenesis. 
Lymphocytic vasculitis may also be seen in rickettsial infections such as 
Rocky Mountain spotted fever. The pathogenesis of granulomatous vasculitis 
remains poorly understood and is thought to be induced by a combination of 
circulating immune complexes and a cell-mediated immune response. 

============================================================ 
43.) Pleural effusions in an overlap syndrome of idiopathic hypereosinophilic syndrome and erythema elevatum diutinum. 
============================================================ 
Eur Respir J 1990 Jan;3(1):115-8 

Cordier JF, Faure M, Hermier C, Brune J 
Dept. of Pneumology, Hopital Louis Pradel, Universite Claude Bernard, Lyon, 
France. 

We report a patient with idiopathic hypereosinophilic syndrome presenting 
with bilateral eosinophilic pleural effusions. He also had erythema 
elevatum diutinum, a rare skin disease of the vasculitic type. No 
cardiomyopathy was present. Pleural effusions, skin lesions, and blood 
eosinophilia disappeared with prolonged corticosteroid treatment. 

============================================================ 
44.) Erythema elevatum diutinum: an ultrastructural case study. 
============================================================ 
J Cutan Pathol 1989 Aug;16(4):211-7 

Lee AY, Nakagawa H, Nogita T, Ishibashi Y 
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan. 

An electron microscopic study was made of a patient with erythema elevatum 
diutinum, a rare variant of cutaneous leukocytoclastic vasculitis. Biopsies 
were obtained from early evolving and late fibroproliferative lesions. 
Early lesions showed vasculitis and a massive dermal infiltrate composed 
mainly of neutrophils, histiocytes/macrophages and apparently increased 
Langerhans cells. Later lesions were characterized by a dense fibrosis with 
a proliferation of fibroblasts and myofibroblasts and a dermal infiltrate 
in which lymphocytes and histiocytes/macrophages predominated. Even in 
later lesions, dermal Langerhans cells were observed in great number. 
Langerhans cells may be involved in the evolution of this rare disease. 

============================================================ 
45.) [Erythema elevatum diutinum--a rare variant of skin changes in systemic 
lupus erythematosus]. 
============================================================ 
Dermatol Monatsschr 1989;175(10):628-34 

Schulze P, Audring H, Sonnichsen N 
It is reported on 5 patients with erythema elevatum diutinum, in 2 of these 
cases exists a systemic lupus erythematosus and another patient suffers 
from cutaneous lupus erythematosus. This information can be considered as a 
possibility that cutaneous alterations similar to erythema elevatum 
diutinum could be the first symptoms of systemic lupus erythematosus when 
the usual treatment is inefficient. 
=================================================================== 
DATA-MEDICOS/DERMAGIC-EXPRESS No 2-(84) 23/11/99 DR. JOSE LAPENTA R. 
=================================================================== 

 

Produced by Dr. Jose Lapenta R. Dermatologist 
                 Maracay Estado Aragua Venezuela 1.999  
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