EDITORIAL ESPANOL 
================= 
Hola amigos de la red, DERMAGIC de nuevo con ustedes, el tema de hoy LA 
PAPULOSIS LINFOMATOIDE, enfermedad de la piel que bien esta demostrado es 
precursora de malignidad (LIMFOMA). Su relacion con la PITIRIASIS LIQUENOIDE 
AGUDA Y CRONICA tambien esta demostrada y bien documentada. El tratamiento, 
dificil. 
Les puedo asegurar que si examinan bien estas fotos su primer diagnostico 
probablemente sea: INFECCION de la piel (IMPETIGO CONTAGIOSO) y su ultimo: 
PAPULOSIS LINFOMATOIDE,,, es todo lo contrario... se trata de una PAPULOSIS 
LINFOMATOIDE. Espero disfruten estas 65 referencias. 

Les recuerdo que dermagic sera quincenal a partir de esta fecha ,,, 

Saludos a todos !!! 

Dr. Jose Lapenta R.,,, 

EDITORIAL ENGLISH 
================= 
Hello friends of the net, DERMAGIC again with you, today's topic THE 
LYMPHOMATOID PAPULOSIS , illness of the skin that is well demonstrated is 
precursor of malignancy (LYMPHOMA). Their relationship with the PITYRIASIS 
LICHENOIDES ACUTE AND CHRONIC also is demonstrated and well documented. 
The treatment, difficult. 
I can assure that if you examine these pictures well your first I diagnose
be probably: 
INFECTION of the skin (IMPETIGO CONTAGIOSA) and their I last: 
LYMPHOMATOID PAPULOSIS, it is just the opposite... it is a LYMPHOMATOID 
PAPULOSIS I wait enjoyments these 65 references. 

I remind that dermagic will be biweekly starting from this date, 

Greetings to ALL, !! 
Dr. Jose Lapenta R.,,, 
=================================================================== 
REFERENCIAS BIBLIOGRAFICAS / BIBLIOGRAPHICAL REFERENCES 
=================================================================== 
================================================================= 
1.) Lymphomatoid papulosis associated with pregnancy. 
2.) Lymphomatoid papulosis: successful weekly pulse superpotent topical 
corticosteroid therapy in three pediatric patients. 
3.) Lymphomatoid papulosis and cutaneous CD30+ lymphoma. 4.) Amplification of 
genomic DNA demonstrates the presence of the t(2;5) (p23;q35) in anaplastic
large cell 
lymphoma, but not in other non-Hodgkin's lymphomas, Hodgkin's disease, or 
lymphomatoid papulosis [see comments] 
5.) A case of lymphomatoid papulosis occurred simultaneously with
Ki-1-positive 
anaplastic large cell lymphoma. 
6.) Lymphomatoid papulosis type A: clinical, morphologic, and
immunophenotypic study. 
7.) Increased risk of lymphoid and nonlymphoid malignancies in patients with 
lymphomatoid papulosis. 
8.) Lymphomatoid papulosis in association with mycosis fungoides: a study
of 15 cases. 
9.) Involvement of the tongue by lymphomatoid papulosis. 
10.) Lymphomatoid papulosis treated with extracorporeal photochemotherapy. 
11.) Absence of anaplastic lymphoma kinase (ALK) and Epstein-Barr virus 
gene products in primary cutaneous anaplastic large cell lymphoma and
lymphomatoid 
papulosis. 
12.) Absence of Epstein-Barr virus in lymphomatoid papulosis. An
immunohistochemical 
and in situ hybridization study [see comments] 
13.) Methotrexate is effective therapy for lymphomatoid papulosis and other
primary 
cutaneous CD30-positive lymphoproliferative disorders. 
14.) Follicular lymphomatoid papulosis. 
15.)Is it lymphoma or lymphomatoid papulosis? 
16.) Lymphomatoid papulosis: a low-grade T-cell lymphoma? 
17.) The dominant T cell clone is present in multiple regressing skin
lesions and associated 
T cell lymphomas of patients with lymphomatoid papulosis. 
18.) The cytokine mRNA expression in primary cutaneous CD30-positive 
lymphoproliferative disorders: successful treatment with recombinant
interferon-gamma. 
19.) Low incidence of Epstein-Barr virus presence in primary cutaneous T-cell 
lymphoproliferations. 
20.) Clonal disease in extracutaneous compartments in cutaneous T-cell 
lymphomas. A comparative study between cutaneous T-cell lymphomas and pseudo 
lymphomas. 
21.) Apoptotic and proliferating cells in cutaneous lymphoproliferative
diseases. 
22.) No evidence of HTLV-I proviral integration in lymphoproliferative
disorders 
associated with cutaneous T-cell lymphoma. 
23.) Interleukin-7 receptor expression in cutaneous T-cell lymphomas. 
24.) The t(2;5) in human lymphomas. 
25.) Pityriasis lichenoides in children: clinicopathologic review of 22
patients. 
26.) Regional lymphomatoid papulosis: a report of four cases. 
27.) Infection with parapoxvirus induces CD30-positive cutaneous
infiltrates in humans. 
28.) CD30-CD30 ligand interaction in primary cutaneous CD30(+) T-cell
lymphomas: A 
clue to the pathophysiology of clinical regression. 
29.) Increased risk of lymphoid and nonlymphoid malignancies in patients with 
lymphomatoid papulosis. 
30.) Primary cutaneous CD8-positive epidermotropic cytotoxic T cell
lymphomas. A 
distinct clinicopathological entity with an aggressive 
clinical behavior. 
31.) Lymphomatoid papulosis in association with mycosis fungoides: a study
of 15 cases. 
32.) Early detection of cutaneous lymphoma. 
33.) Cutaneous pseudolymphomas. 
34.) Lymphomatoid papulosis in childhood with exclusive acral involvement. 
35.) Absence of HTLV-1 proviral sequences in patients with lymphomatoid
papulosis 
36.) Clinicopathologic manifestations of Epstein-Barr virus-associated
cutaneous 
lymphoproliferative disorders. 
37.) Follicular lymphomatoid papulosis and multiple myeloma. 
38.) Lymphomatoid papulosis--treatment with recombinant interferon alfa-2a
and 
etretinate. 
39.) Lymphomatoid papulosis: a follow-up study of 41 patients. 
40.) Lymphomatoid papulosis associated with acquired ichthyosis. 
41.) Primary anaplastic large-cell lymphoma of the skin. A case report
suggesting that 
regressing atypical histiocytosis and lymphomatoid papulosis are subsets. 
42.) Lymphomatoid papulosis: a clinical and histopathologic review of 53
cases with 
leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene 
rearrangement studies. 
43.) [Lymphomatoid papulosis and anaplastic giant-cell lymphoma] 
44.) Molecular evidence for a clonal relationship between lymphomatoid
papulosis and 
Ki-1 positive large cell anaplastic lymphoma. 
45.) Lymphomatoid papulosis followed by Hodgkin's lymphoma. Differential
response to 
therapy [see comments] 
46.) Epidemiology of lymphomatoid papulosis [see comments] 
47.) Lethal midline granuloma (peripheral T-cell lymphoma) after lymphomatoid 
papulosis. 
48.) The prognosis of patients with lymphomatoid papulosis associated with
malignant 
lymphomas. 
49.) Immunophenotypic and genotypic characterization of lymphomatoid
papulosis. 
50.) Pityriasis lichenoides and lymphomatoid papulosis. 
51.) Lymphomatoid papulosis: clinicopathological comparative study with
pityriasis 
lichenoides et varioliformis acuta. 
52.) PUVA-induced lymphomatoid papulosis in a patient with mycosis fungoides. 
53.) Accuracy in diagnosis of lymphomatoid papulosis. 
54.)Regressing atypical histiocytosis and lymphomatoid papulosis: variants
of the same 
disorder? 
55.) Lymphomatoid papulosis and its relationship to "idiopathic"
hypereosinophilic 
syndrome. 
56.) Lymphomatoid papulosis/pityriasis lichenoides in two children. 
57.) [Acyclovir in mycosis fungoides and lymphomatoid papulosis] 
58.) Eosinophilic histiocytosis: a variant form of lymphomatoid papulosis
or a disease sui 
generis? 
59.) Lymphomatoid papulosis: a premalignant T cell disorder. 
60.) Clinical and histologic differentiation between lymphomatoid papulosis
and pityriasis 
lichenoides. 
61.) The clinicopathologic spectrum of lymphomatoid papulosis: study of 31
cases. 
62.) [Lymphomatoid papulosis in a child] 
63.)Lymphomatoid papulosis in an 11-month-old infant. 
64.) Topical carmustine therapy for lymphomatoid papulosis. 
65.)Immunohistology of pityriasis lichenoides et varioliformis acuta and
pityriasis 
lichenoides chronica. Evidence for their interrelationship with
lymphomatoid papulosis. 
============================================================ 
============================================================ 
1.) Lymphomatoid papulosis associated with pregnancy. 
============================================================ 
Author 
Yamamoto O; Tajiri M; Asahi M 
Address 
Department of Dermatology and Occupational Dermatopathology, University of 
Occupational and Environmental Health, Kitakyushu, Japan. 
Source 
Clin Exp Dermatol, 22(3):141-3 1997 May 
Abstract 
We report a case of lymphomatoid papulosis which developed in a 29-year-old 
pregnant woman. She had numerous papules scattered over the inner aspect of 
the left thigh. Histology of the biopsy specimen demonstrated an atypical 
mononuclear cell infiltration of the dermis. Spontaneous regression of the 
lesions occurred after termination of gestation. A possible effect of 
hormonal changes and alterations in T lymphocyte activity during pregnancy 
on the occurrence of lymphomatoid papulosis is discussed. In 1968, Macaulay 
introduced the term lymphomatoid papulosis for a chronic self-healing skin 
lesion which was clinically benign and histologically malignant. 
Clinically, lymphomatoid papulosis consists of involuting and recurring 
papules, plaques and nodules. Histopathologically, the lesion is 
characterized by an atypical lymphoid infiltrate which resembles malignant 
lymphoma. Immunohistochemically, the atypical lymphoid cells bear T-cell 
markers and are characterized by the expression of Ki-1 or CD30. We 
describe the first case of typical lymphomatoid papulosis which developed 
during pregnancy. 

============================================================ 
2.) Lymphomatoid papulosis: successful weekly pulse superpotent topical 
corticosteroid therapy in three pediatric patients. 
============================================================ 
Author 
Paul MA; Krowchuk DP; Hitchcock MG; Jorizzo JL 
Address 
Department of Dermatology, Bowman Gray School of Medicine, Wake Forest 
University, Winston-Salem, NC 27157, USA. 
Source 
Pediatr Dermatol, 13(6):501-6 1996 Nov-Dec 
Abstract 
Lymphomatoid papulosis is a T-cell proliferation that occurs primarily in 
adults but has been well described in children. Lesions may regress 
spontaneously but often leave residual scarring and, as a result, 
intervention frequently is considered. Therapeutic modalities commonly 
employed for adults with lymphomatoid papulosis may be poorly tolerated by 
pediatric patients. We present a series of three children with lymphomatoid 
papulosis treated with superpotent topical corticosteroids (halobetasol or 
clobetasol propionate). When applied twice daily for 2 to 3 weeks followed 
by weekly pulsed application, this treatment resulted in complete 
resolution of nearly all cutaneous lesions. Three ulcerated lesions, 
occurring in two patients, required adjuvant therapy with intralesional 
triamcinolone. To date one patient remains free of cutaneous disease and 
two children experience occasional new lesions that respond to renewed 
treatment with topical clobetasol propionate. None of the children have 
evidence of systemic disease. We conclude that pulsed application of a 
superpotent topical corticosteroid is efficacious and safe in the 
management of cutaneous lesions of lymphomatoid papulosis and avoids the 
risks often associated with more aggressive interventions. Since these 
agents do not alter the risk of subsequent malignancy, careful ongoing 
surveillance of children with lymphomatoid papulosis is imperative. 

============================================================ 
3.) Lymphomatoid papulosis and cutaneous CD30+ lymphoma. 
============================================================ 
Author 
LeBoit PE 
Address 
Department of Pathology, University of California, San Francisco 
94143-0506, USA. 
Source 
Am J Dermatopathol, 18(3):221-35 1996 Jun 
Abstract 
Lymphomatoid papulosis and cutaneous CD30+ lymphoma are closely related 
conditions in which large atypical lymphocytes that have similar 
immunophenotypic features occur. In lymphomatoid papulosis, the lesions are 
papules and nodules that spontaneously involute. There are two polar 
histologic patterns, type A and B, in which the large atypical cells 
resemble those of Hodgkin's disease and mycosis fungoides, respectively, 
but in many cases, features of both types are present, either separately or 
in the same lesions. Variants of lymphomatoid papulosis include cases with 
a perifollicular distribution and those with lymphocytic vasculitis or 
dermal mucin deposits. Clinical lesions that tend to be stable, a 
monomorphous cellular composition, and in the case of immunocompromised 
patients, the presence of Epstein-Barr viral genome characterize cutaneous 
CD30+ lymphoma. A loss of response to transforming growth factor-beta, 
which normally dampens cellular proliferation, may differentiate CD30+ 
lymphoma from lymphomatoid papulosis. 

============================================================ 
4.) Amplification of genomic DNA demonstrates the presence of the t(2;5) 
(p23;q35) in anaplastic large cell lymphoma, but not in other non-Hodgkin's 
lymphomas, Hodgkin's disease, or lymphomatoid papulosis [see comments] 
============================================================ 
Author 
Sarris AH; Luthra R; Papadimitracopoulou V; Waasdorp M; Dimopoulos MA; 
McBride JA; Cabanillas F; Duvic M; Deisseroth A; Morris SW; Pugh WC 
Address 
Department of Hematology, University of Texas M.D. Anderson Cancer Center, 
Houston 77030, USA. 
Source 
Blood, 88(5):1771-9 1996 Sep 1 
Abstract 
Anaplastic large cell lymphoma (ALCL) is a distinct clinicopathologic 
variant of intermediate grade non-Hodgkin's lymphomas (NHL) composed of 
large pleomorphic cells that usually express the CD30 antigen and 
interleukin (IL)-2 receptors, and is characterized by frequent cutaneous 
and extranodal involvement. With variable frequency ALCL bear the 
t(2;5)(p23;q35) chromosomal translocation that fuses the nucleophosmin 
(NPM) gene on chromosome 5q35 to a novel protein kinase gene, Anaplastic 
Lymphoma Kinase (ALK), on chromosome 2p23. We determined the frequency of 
this translocation with a novel DNA polymerase chain reaction (PCR) 
technique using 0.5 microgram of genomic DNA, 5'-primers derived from the 
NPM gene and 3'-primers derived from the ALK gene and hybridization with 
internal probes. The presence of amplifiable DNA in the samples was tested 
with the inclusion in the PCR reaction of oligonucleotide primers designed 
to amplify a 3016-bp fragment from the beta-globin locus. NMP-ALK fusion 
amplicons were detected using DNA isolated either from all three ALCL cell 
lines tested, or from all four primary ALCL tumors known to contain the 
t(2;5)(p23;q35) translocation. Nested amplicons were detected by 
hybridization in 100% of specimens diluted 10(4)-fold and in 20% of those 
diluted 10(5)-fold. We subsequently examined archival genomic DNA from 20 
patients with ALCL, 39 with diffuse large cell, 2 with mantle cell, 20 with 
peripheral T cell, 13 with low-grade NHL, 31 with Hodgkin's disease (HD), 
and 6 with lymphomatoid papulosis. Fusion of the NPM and ALK genes was 
detected in three of 18 patients with ALCL who had amplifiable DNA (17%, 
95% confidence intervals 4% to 41%), but not in any patients with other 
NHL, HD, or lymphomatoid papulosis. The amplicon sizes were different in 
all cell lines and patients reflecting unique genomic DNA breakpoints. We 
conclude that with genomic DNA-PCR the rearrangement of the NPM and ALK 
loci is restricted to patients with ALCL. Further studies are needed to 
determine the prognostic significance of the NPM-ALK rearrangement, to 
determine whether its detection can aid in the differential diagnosis 
between ALCL. Hodgkin's disease, and lymphomatoid papulosis, and to 
establish the usefulness of the genomic DNA PCR in the monitoring of 
minimal residual disease in those patients whose tumors bear the t(2;5). 

============================================================ 
5.) A case of lymphomatoid papulosis occurred simultaneously with 
Ki-1-positive anaplastic large cell lymphoma. 
============================================================ 
Author 
Lee NS; Cha SW; Hong SJ; Shin WY; Lee GT; Jeon JW; Won JH; Baick SH; Hong 
DS; Park HS 
Address 
Department of Internal Medicine, Soonchunhyang University, College of 
Medicine, Seoul, Korea. 
Source 
Korean J Intern Med, 12(1):84-8 1997 Jan 
Abstract 
Lymphomatoid papulosis (LyP) is a chronic self-healing skin eruption that 
is clinically benign but histologically mimics a malignant lymphoma. 
However, lymphomatoid papulosis with anaplastic large cell lymphoma 
responds poorly to medical treatments, including chemotherapies. We 
experienced a 60-year-old male patient with lymphomatoid papulosis occurred 
simultaneously with relapsed Ki-1-positive anaplastic large cell lymphoma 
who was treated with salvage chemotherapy but, unfortunately, failed to be 
rescued. We report it with a review of the literature. 

============================================================ 
6.) Lymphomatoid papulosis type A: clinical, morphologic, and 
immunophenotypic study. 
============================================================ 
Author 
Sioutos N; Asvesti C; Sivridis E; Aygerinou G; Tsega A; Zakopoulou N; 
Zographakis I 
Address 
Department of Pathology, Georgetown University, Washington, DC, USA. 
Source 
Int J Dermatol, 36(7):514-7 1997 Jul 
Abstract 
BACKGROUND: Lymphomatoid papulosis (LyP) is a cutaneous clonal or 
polyclonal Ki-1 + T-cell lymphoproliferative disorder, morphologically 
resembling Ki-1 + anaplastic large cell lymphomas (Ki-1 + ALCL) or 
Hodgkin's disease (HD). Lymphomatoid papulosis usually has a characteristic 
benign clinical course with remissions and relapses of the cutaneous 
eruptions. METHODS: The authors studied three patients with LyP. In each 
case the diagnosis was established based on the typical clinical history 
and presentation of the cutaneous lesions as well as the morphologic and 
immunophenotypic findings. RESULTS: In all three cases the skin biopsies 
showed a polymorphic, nonepidermotropic, dermal lymphocytic infiltrate, 
composed of small lymphocytes and fewer large, atypical cells. The large 
cells were positive for the activation markers CD30 (Ki-1) and CD45R 
(leukocyte common antigen), and were negative for the HD marker CD15 (Leu 
MI). CONCLUSIONS: In most cases, LyP can be distinguished from Ki-1 + ALCL 
and HD on the basis of clinical, morphologic, and/or immunophenotypic 
findings. We emphasize the importance of the recognition of LyP as a 
clinicopathologic entity and the awareness of dermatologists, oncologists, 
and surgical pathologists in differentiating LyP from other primary 
cutaneous Ki-1 + lymphoproliferative disorders (Ki-1 + ALCL and HD). The 
prognosis of cutaneous Ki-1 + ALCL and HD is usually different from LyP and 
requires a different therapeutic approach. 

============================================================ 
7.) Increased risk of lymphoid and nonlymphoid malignancies in patients 
with lymphomatoid papulosis. 
============================================================ 
Author 
Wang HH; Myers T; Lach LJ; Hsieh CC; Kadin ME 
Address 
Department of Pathology, Beth Israel Deaconess Medical Center and Harvard 
Medical School, Boston, Massachusetts 02115, USA. 
Source 
Cancer, 86(7):1240-5 1999 Oct 1 
Abstract 
BACKGROUND: Lymphomatoid papulosis (LyP) is a rare skin disease with 
malignant potential. The long term outcomes of patients with this disease 
have not been adequately assessed. METHODS: Fifty-seven patients with 
biopsy-proven LyP and 67 controls matched for age, gender, and race were 
followed prospectively from 1988 to 1996. Reported malignancies were 
confirmed by surgical pathology and/or autopsy reports. A search through 
the National Death Index through December 1995 was conducted to identify 
all deaths, and death certificates were procured. Expected numbers of 
malignancies based on SEER data were calculated for both the patient and 
the control groups. RESULTS: Six LyP patients (10.5%) and 1 control (1.5%) 
reported nonlymphoid malignancies (P = 0.047). Two patients and no controls 
developed lymphoid malignancies (mycosis fungoides and CD30(+) cutaneous 
lymphoma). The expected numbers of nonlymphoid and lymphoid malignancies in 
the LyP patient group, based on the SEER data, were 1.93 and 0.15, 
respectively, yielding a relative risk (with 95% confidence interval) of 
3.11 (1.26-6.47) for nonlymphoid malignancies and 13.33 (2.24-44.05) for 
malignant lymphomas in the LyP patients. There was no significant 
difference between the observed and expected numbers of malignancies in the 
control group. Four LyP patients died during the follow-up, three due to 
malignancies; and one control died of a gunshot wound to the head 
(suicide). The difference in overall survival between the LyP patients and 
the controls was not statistically significant (P = 0. 12). CONCLUSIONS: 
Patients with LyP appear to have an increased risk of both lymphoid and 
nonlymphoid malignancies. The increased risk of nonlymphoid as well as 
lymphoid malignancies may suggest a basic underlying genetic defect leading 
to the development of malignancy in LyP patients. Copyright 1999 American 
Cancer Society. 

============================================================ 
8.) Lymphomatoid papulosis in association with mycosis fungoides: a study 
of 15 cases. 
============================================================ 
Author 
Basarab T; Fraser-Andrews EA; Orchard G; Whittaker S; Russel-Jones R 
Address 
St John's Institute of Dermatology, St Thomas' Hospital, London, UK. 
Source 
Br J Dermatol, 139(4):630-8 1998 Oct 
Abstract 
We report clinical findings in 15 patients with lymphomatoid papulosis 
(LyP) associated with mycosis fungoides (MF). LyP either preceded (n = 4), 
followed (n = 5) or occurred concurrently with the MF lesions (n = 6). 
Twenty-eight LyP lesions were classified histologically and analysed 
further with immunostaining for CD3 and CD30. Five biopsies contained a 
predominance of type A cells, six biopsies contained a predominance of type 
B cells. and six were mixed (A + B). However, 11 biopsies contained a 
population of atypical mononuclear cells with large hyperchromatic nuclei 
that we have termed indeterminate cells. These cells contained a thin rim 
of eosinophilic cytoplasm and showed strong CD30 but absent, faint or 
normal CD3 staining. In seven biopsies from five separate patients these 
cells represented the predominant cell type and we have termed this the 
pleomorphic variant of LyP. Analysis of T-cell receptor genes using 
Southern blot analysis and polymerase chain reaction/single strand 
conformational polymorphism analysis identified a T-cell clone in six of 16 
LyP lesions and nine of 16 MF lesions. In the three patients who had clones 
in both types of skin lesions, the clones were identical. Only two of 10 
blood samples, both of which were from the same patient, had a T-cell clone 
and none of two lymph nodes showed evidence of a clonal population. To date 
all patients are alive with a median follow-up of 15 years from the onset 
of the first lesion. One patient has developed Lin anaplastic large cell 
lymphoma of the nasopharynx. These data augment the current literature on 
the association of LyP and MF and suggest that the 

============================================================ 
9.) Involvement of the tongue by lymphomatoid papulosis. 
============================================================ 
Author 
Kato N; Tomita Y; Yoshida K; Hisai H 
Address 
Department of Dermatology and Research Institute, National Sapporo 
Hospital, Japan. 
Source 
Am J Dermatopathol, 20(5):522-6 1998 Oct 
Abstract 
We report on a case of lymphomatoid papulosis (LyP) with involvement of the 
tongue. The patient was a 34-year-old Japanese man. Three reddish, 
centrally depressed, slightly elevated nodules were evident on the dorsal 
tongue, along with lesions elsewhere on the skin. One of them was biopsied 
and exhibited a superficial and deep, perivascular and interstitial mixed 
cellular infiltrate including atypical lymphoid cells, lymphocytes, 
neutrophils, and histiocytes. The patient also showed rhythmical recurrence 
of reddish papules and ulcerated nodules on the trunk, extremities, and 
anogenital area. Histologically, these papules showed a dense, wedge-shaped 
mixed cellular infiltrate in the dermis, which included medium and large 
atypical lymphoid cells, lymphocytes, neutrophils, and histiocytes. 
Immunoperoxidase staining for CD30 was positive in the cell membrane and 
cytoplasm of the atypical cells. We could not find other reports of LyP 
involving the tongue. Systemic treatment with interferon (INF)-alpha2a was 
dramatically effective in inhibiting recurrence of the eruption. 

============================================================ 
10.) Lymphomatoid papulosis treated with extracorporeal photochemotherapy. 
============================================================ 
Author 
Wollina U 
Address 
Department of Dermatology, University of Jena, 07740 Jena, Germany. 
Source 
Oncol Rep, 5(1):57-9 1998 Jan-Feb 
Abstract 
Lymphomatoid papulosis (LP) is a rare low-grade T-cell lymphoma which may 
respond to cytotoxic drugs and PUVA irradiation but long-term remission has 
not been achieved. Extracorporeal photochemotherapy (ECP) is an 
immunomodulating therapy used successfully for several types of CTCL, no 
experience with LP has been reported yet. ECP therapy with 
8-methoxypsoralen was introduced on two subsequent days once per month for 
half a year in a 42-year old women with a 20-year history of LP. 
Disseminated papules disappeared rapidly after 3 cycles of ECP treatment 
but metastatic spread continued, which made necessary a subsequent 
polychemotherapy. One year later, the patient died of central nervous 
system metastasis. ECP monotherapy seems unable to control disease 
progression in LP despite beneficial effects on skin lesions. 

============================================================ 
11.) Absence of anaplastic lymphoma kinase (ALK) and Epstein-Barr virus 
gene products in primary cutaneous anaplastic large cell lymphoma and 
lymphomatoid papulosis. 
============================================================ 
Author 
Herbst H; Sander C; Tronnier M; Kutzner H; H¨ugel H; Kaudewitz P 
Address 
Institut f¨ur Pathologie, Universit¨atsklinikum Benjamin Franklin, Freie 
Universit¨at, Berlin, Germany. 
Source 
Br J Dermatol, 137(5):680-6 1997 Nov 
Abstract 
The prevalence of the t(2;5)(p23;q35) and/or anaplastic lymphoma kinase 
(ALK) gene products in cutaneous anaplastic large cell (ALC) lymphomas and 
a potential precursor lesion, lymphomatoid papulosis (LyP), is 
controversial. ALK gene products, which are absent from normal 
lymphohaematopoietic cells, are a phenotypic marker of lymphomas carrying 
the t(2;5). We used in situ hybridization and immunohistology to screen 14 
cutaneous ALC lymphomas, 21 cases of LyP, and one nodal ALC lymphoma 
associated with LyP for ALK gene products. ALK gene products were not 
detectable in these cases. In contrast, ALK gene products were found in a 
lymphonodal ALC lymphoma with subsequent extension to the skin and in 
t(2;5)-positive cell lines. Detection of the Epstein-Barr virus 
(EBV)-encoded small nuclear transcripts (EBER), and of immunoglobulin light 
chain transcripts served to check for the presence of cellular RNA in the 
tissue sections. EBER transcripts were found in scattered reactive lymphoid 
cells, but not in atypical or tumour cells. ALK gene expression and EBV 
infection seem to be a rare finding in cutaneous ALC lymphomas and LyP. 
This points to a molecular aetiology of primary cutaneous ALC lymphomas and 
LyP distinct from that of extracutaneous CD30+ lymphoproliferative disease. 
Detection of the t(2;5) or ALK gene products in cutaneous 
lymphoproliferative lesions therefore requires exclusion of extracutaneous 
ALC lymphoma in such patients. 

============================================================ 
12.) Absence of Epstein-Barr virus in lymphomatoid papulosis. An 
immunohistochemical and in situ hybridization study [see comments] 
============================================================ 
Author 
Sang¨ueza OP; Galloway J; Eagan PA; Braziel RM; Gulley ML 
Address 
Department of Pathology and Medicine, Medical College of Georgia, Augusta, 
USA. 
Source 
Arch Dermatol, 132(3):279-82 1996 Mar 
Abstract 
BACKGROUND AND DESIGN: Lymphomatoid papulosis (LyP) and cutaneous 
Hodgkin's 
disease share many clinical, histopathologic, and immunohistochemical 
features. Epstein-Barr virus (EBV) has been implicated in the pathogenesis 
of several lymphoid malignancies, including Hodgkin's disease. Given the 
similarities between LyP and Hodgkin's disease, we asked if EBV could be 
detected in lesions of LyP. We examined 31 specimens of LyP that were 
obtained from 24 patients for evidence of EBV by in situ hybridization to 
EBER1 transcripts and for immunohistochemistry of viral latent membrane 
protein 1 (LMP1). RESULTS: In no instance there was there any evidence of 
EBV gene products by either in situ hybridization or immunohistochemistry. 
CONCLUSIONS: The absence of EBV in LyP suggests that this virus is not 
operative in the pathogenesis of LyP. Furthermore, it suggests that LyP and 
Hodgkin's disease may not share the same molecular mechanisms despite their 
phenotypic similarities. 

============================================================ 
13.) Methotrexate is effective therapy for lymphomatoid papulosis and other 
primary cutaneous CD30-positive lymphoproliferative disorders. 
============================================================ 
Author 
Vonderheid EC; Sajjadian A; Kadin ME 
Address 
Department of Dermatology, Medical College of Pennsylvania, Philadelphia, 
USA. 
Source 
J Am Acad Dermatol, 34(3):470-81 1996 Mar 
Abstract 
BACKGROUND: The spectrum of primary cutaneous CD30+ lymphoproliferative 
disease consists of lymphomatoid papulosis (LyP) at one extreme and CD30+ 
peripheral T-cell lymphoma (Ki-1+ lymphoma) presenting in the skin at the 
other extreme. Methotrexate has been reported to be effective in LyP, but 
the experience has been limited to single case reports or small series. 
OBJECTIVE: The objective was to determine the effectiveness of methotrexate 
in the treatment of primary cutaneous DC30+ lymphoproliferative disease. 
METHODS: We reviewed our 20-year experience with the use of methotrexate in 
45 patients with relatively severe LyP, Ki-1+ lymphoma, and interface 
presentations. RESULTS: During induction of methotrexate therapy patients 
received maximum doses ranging from 10 to 60 mg/week (median, 20 mg/week). 
Clinical improvement usually occurred quickly, typically at doses of 15 to 
20 mg weekly, and satisfactory long-term control was achieved in 39 
patients (87%) with maintainance doses given at 10 to 14-day intervals 
(range, 7 to 28 days). After methotrexate was discontinued, 10 patients 
remained free of CD30+ lesions from more than 24 months to more than 227 
months (median, more than 127 months). The median total duration of 
methotrexate therapy for all patients exceeded 39 months (range, 2 to 205 
months). Adverse effects were generally mild and transient and included 
fatigue (47%), nausea (22%), weight loss (13%), diarrhea or 
gastrointestinal cramping (10%), increased serum hepatic transaminase 
levels (27%), anemia (11%), or leukopenia (9%). Early hepatic fibrosis was 
found in 5 of 10 patients, all of whom had been treated for more than 3 
years (range, 38 to 111 months). CONCLUSION: Low-dose methotrexate (25 mg 
or less given at 1-to 4-week intervals) is an effective and well-tolerated 
treatment of selected patients with primary cutaneous CD30+ 
lymphoproliferative disease. 

============================================================ 
14.) Follicular lymphomatoid papulosis. 
============================================================ 
Author 
Kato N; Matsue K 
Address 
Department of Dermatology, Hokkaido University School of Medicine, Japan. 
Source 
Am J Dermatopathol, 19(2):189-96 1997 Apr 
Abstract 
A case of follicular lymphomatoid papulosis (LyP) is reported. The patient 
was a 60-year-old Japanese woman. Clinically, cutaneous eruptions were 
reddish, centrally depressed, dome-shaped papules on the extensor aspect of 
the forearm. Histologically, they exhibited features that fulfilled the 
disease criteria described by Pierard, et al., i.e., (Am J Dermatopathol 
1980;2:173-80), mixed cellular infiltrates including atypical 
Reed-Sternberg cell-like type-A cells and mycosis cell-like type-B cells 
surrounding hyperplastic follicular epithelia. The patient also showed many 
typical nonfollicular LyP papules, i.e., rhythmically recurrent papules 
which underwent spontaneous involution within a few weeks, over a 10-year 
period. The coincidental occurrence of a rare variant of follicular LyP and 
typical LyP in the same individual further suggests that follicular LyP is 
merely a histological pattern of LyP involving epithelial adnexae. 

============================================================ 
15.)Is it lymphoma or lymphomatoid papulosis? 
============================================================ 
Author 
Demierre MF; Goldberg LJ; Kadin ME; Koh HK 
Address 
Department of Dermatology, Boston University School of Medicine, MA, USA. 
Source 
J Am Acad Dermatol, 36(5 Pt 1):765-72 1997 May 
Abstract 
Distinguishing malignancy from premalignant conditions can be difficult. 
Controversy surrounds both the clinical and histologic criteria used to 
distinguish lymphomatoid papulosis, a benign disorder, from CD30+ 
anaplastic large-cell lymphoma. Three case histories illustrate important 
points in categorizing different lymphoproliferative disorders as benign or 
malignant. We emphasize a multidisciplinary approach to improve diagnosis 
and patient management. 

============================================================ 
16.) Lymphomatoid papulosis: a low-grade T-cell lymphoma? 
============================================================ 
Author 
Orchard GE 
Address 
Dermatopathology Department, St Thomas' Hospital, London, England. 
Source 
Br J Biomed Sci, 53(2):162-9 1996 Jun 
Abstract 
Lymphomatoid papulosis is a chronic cutaneous lymphoid disease 
characterised clinically by the presence of recurrent papulonodular or 
plaque like lesions, which appear benign. Paradoxically, histological and 
cytopathological features demonstrate features of malignancy. This 
annotation highlight the current theories and technical advances into the 
assessment of this condition, with emphasis on possible pathogenic disease 
mechanisms. 

============================================================ 
17.) The dominant T cell clone is present in multiple regressing skin 
lesions and associated T cell lymphomas of patients with lymphomatoid 
papulosis. 
============================================================ 
Author 
Chott A; Vonderheid EC; Olbricht S; Miao NN; Balk SP; Kadin ME 
Address 
Department of Pathology, Beth Israel Hospital, Boston, Massachusetts, USA. 
Source 
J Invest Dermatol, 106(4):696-700 1996 Apr 
Abstract 
This study was undertaken to determine the clonality of lymphomatoid 
papulosis (LyP), its clonal relationship to lymphomas, which occur at high 
frequency in LyP patients, and to define the cell lineage of 
Reed-Sternberg-like cells in type A lesions of LyP. Punch biopsies of skin 
of 11 adult patients with LyP were analyzed for morphologic subtype of LyP, 
surface antigens, and clonal T-cell receptor (TCR) gene rearrangements. 
Clonal rearrangements were identified by semiquantitative polymerase chain 
reaction amplification and sequencing of TCR-beta chain genes in nine 
patients and TCR-gamma chain genes in two patients. A single dominant clone 
was detected in multiple separate LyP lesions, often of different 
histologies, in nine patients. The same clone was detected in LyP lesions 
and the anaplastic large cell lymphoma (ALCL) of 2 patients and the mycosis 
fungoides (MF) of 2 other patients. No dominant clone could be detected in 
one patient with LyP uncomplicated by lymphoma or in a second patient with 
LyP and MF. A T-cell lineage was evident for RS-like cells in cell culture 
and in type A lesions. These results show that multiple regressing skin 
lesions and associated T cell lymphomas (MF and ALCL) are clonally related 
in most LyP patients, which suggest that the disease in these patients was 
initiated by a non-random genetic event. 

============================================================ 
18.) The cytokine mRNA expression in primary cutaneous CD30-positive 
lymphoproliferative disorders: successful treatment with recombinant 
interferon-gamma. 
============================================================ 
Author 
Yagi H; Tokura Y; Furukawa F; Takigawa M 
Address 
Department of Dermatology, Hamamatsu University School of Medicine, Japan. 
Source 
J Invest Dermatol, 107(6):827-32 1996 Dec 
Abstract 
Primary cutaneous CD30 (Ki-1)+ large cell lymphoma (KiL) and lymphomatoid 
papulosis (LyP) type A are collectively termed as primary cutaneous 
CD30-positive lymphoproliferative disorders. We examined the cytokine 
profile of skin-infiltrating cells and the therapeutic efficacy of 
recombinant interferon-gamma (rIFN-gamma) in primary cutaneous KiL and LyP 
type A. By reverse transcriptase-polymerase chain reaction, mRNAs for 
interleukin-4 (IL-4) and IL-10 were detected in the dermis of skin lesions 
in all cases (three cases of KiL and four cases of LyP). In addition, 
tissue from one KiL patient transcribed IL-2 and IFN-gamma messages, and 
one LyP patient showed IL-2 mRNA. In contrast, normal skin from ten healthy 
donors contained mRNA for IL-2 or IFN-gamma, or both, but not for IL-4. 
Before the therapeutic trial of rIFN-gamma, the response of skin lesions 
was assessed by a predictive skin test with local injection of rIFN-gamma 
(0.5 x 10(6) Japan Reference Units [JRU; 1 JRU roughly corresponds to 4 NIH 
units]) for 3 consecutive days in two KiL and two LyP patients. Numbers of 
skin-infiltrating CD30+ cells were decreased, and transcription of mRNA for 
IL-4 and IL-10 was downregulated after the skin test in one KiL and two LyP 
cases. One KiL patient showed no histologic response or change in mRNA 
expression. In the therapeutic trial, rIFN-gamma (total doses of 1.2-4.0 x 
10(7) JRU) was administered intravenously (n = 2) or locally (n = 2). In 
three patients who responded to the skin test, the lesions were objectively 
improved and the numbers of skin-infiltrating CD30+ cells were markedly 
decreased after the therapeutic trial. No improvement was observed in one 
KiL patient who did not respond to the skin test. These findings suggest 
that the skin-infiltrating CD30+ cells in KiL and LyP have a Th2 cytokine 
profile and raise the possibility that the administration of rIFN-gamma 
improves the conditions by inhibiting cytokine mRNA transcription and 
proliferation of CD30+ cells. 

============================================================ 
19.) Low incidence of Epstein-Barr virus presence in primary cutaneous 
T-cell lymphoproliferations. 
============================================================ 
Author 
Anagnostopoulos I; Hummel M; Kaudewitz P; Korbjuhn P; Leoncini L; Stein H 
Address 
Klinikum Benjamin Franklin, Free University Berlin, Germany. 
Source 
Br J Dermatol, 134(2):276-81 1996 Feb 
Abstract 
Multiple biopsies taken from 76 European human immunodeficiency virus 
(HIV)-negative patients with primary cutaneous T-cell lymphoproliferations, 
including mycosis fungoides (MF), pleomorphic T-cell lymphoma (PMTCL), 
anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis (LyP) were 
investigated for the presence of Epstein-Barr virus (EBV) through a 
combined approach. Polymerase chain reaction (PCR) was employed for EBV-DNA 
detection, in situ hybridization (ISH) for cellular localization of 
EBV-encoded nuclear RNAs (EBER1 and EBER2) and immediate early Bam 
H-fragment; lower frame (BHLF) RNA, and immunohistology (IH) for the 
identification of EBV-encoded latent membrane protein 1 (LMP1) and of 
nuclear antigen (EBNA) 2 expression. EBV-DNA was detectable by PCR in 15 of 
76 cases (19.7%). EBER-ISH combined with IH identified a variable, usually 
very low, number of infected neoplastic cells in only seven of the 15 
EBV-DNA-harbouring cases. This discrepancy between the results obtained 
with PCR and ISH is apparently caused by the low number of the infected 
cells per tissue section. The PMTCL entity produced the greatest number of 
positive cases, whilst ALCL and LyP cases were almost constantly devoid of 
the virus. BHLF transcripts were not detectable in any case, nor did any of 
the EBER-positive cells show an LMP1 or EBNA2 expression. These data show 
that primary cutaneous T-cell lymphoproliferations display an infrequent 
association with a latent EBV infection and that the pathogenic role of the 
virus in the positive cases remains obscure as the virus frequently infects 
only a minority of the atypical cells. 

============================================================ 
20.) Clonal disease in extracutaneous compartments in cutaneous T-cell 
lymphomas. A comparative study between cutaneous T-cell lymphomas and 
pseudo lymphomas. 
============================================================ 
Author 
Dommann SN; Dommann-Scherrer CC; Dours-Zimmermann MT; Zimmermann DR; 
Kural-Serbes B; Burg G 
Address 
University Hospital of Z¨urich, Department of Dermatology, Switzerland. 
Source 
Arch Dermatol Res, 288(4):163-7 1996 Apr 
Abstract 
Extracutaneous involvement is a sign of poor prognosis in cutaneous T-cell 
lymphomas (CTCL). Unfortunately it becomes clinically and histologically 
manifest only late in the course of the disease. It was the purpose of this 
study to detect clonality in peripheral blood, lymph nodes and bone marrow 
samples at times when extracutaneous involvement cannot otherwise be 
demonstrated. In addition to skin biopsies, peripheral blood, lymph node 
and bone marrow samples from a total of 25 patients were analysed by 
Southern blotting for clonal gene rearrangement of the T-cell receptor 
beta-chain. Six of the patients were suffering from mycosis fungoides (MF), 
four from non-MF CTCL (pleomorphic T-cell lymphomas), seven from S´ezary 
syndrome (SS), eight from pseudolymphoma (insect bites) (PSL), and one from 
lymphomatoid papulosis (LP). Clonal TcR b gene rearrangements were found in 
patients with MF in four of five skin probes as well as in two of two lymph 
node samples and in one of two peripheral blood samples. In SS patients, 
all skin probes (seven of seven), lymph node samples (six of six), 
peripheral blood samples (six of six) and one bone marrow specimen had a 
clonal TcR beta gene rearrangement. In patients with non-MF CTCL, two of 
four skin, zero of two peripheral blood and one of one bone marrow samples 
with clonal T cells were detected. All investigated patients showed exactly 
the same rearrangement pattern at extranodal sites and in the skin, which 
is proof for the same clone in all compartments. In contrast, no 
rearrangements were detected in LP and PSL (zero of eight skin probes, zero 
of two peripheral blood samples). Our results provide strong evidence for 
an early systemic spread of neoplastic cells in CTCL. However, an initial 
tumour burden has to be reached in order to lead to a clinically and 
prognostically relevant manifestation. 

============================================================ 
21.) Apoptotic and proliferating cells in cutaneous lymphoproliferative 
diseases. 
============================================================ 
Author 
Kikuchi A; Nishikawa T 
Address 
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan. 
Source 
Arch Dermatol, 133(7):829-33 1997 Jul 
Abstract 
BACKGROUND: The cell production vs the cell loss rate in one of the most 
important parameters in evaluating growth and biological behavior of 
neoplasms. Individual cell disintegration in tissues, apoptosis, is a 
constant finding in various tumors and has been shown, by using several 
techniques, as a recognizable cell death that is different from necrosis. 
DESIGN: We studied the apoptosis-proliferation ratio in various 
lymphoproliferative disorders in the skin, including mycosis fungoides 
(MF), cutaneous T-cell lymphoma showing solid tumor mass (CTCL), B-cell 
lymphoma of the skin (BCL), lymphomatoid papulosis (LyP), and cutaneous 
pseudolymphoma by using terminal deoxyuridine triphosphate (dUTP)-biotin 
nick end labeling (TUNEL), a newly developed method to detect 
internucleosomal breaks characteristic of apoptotic cells. SETTING: 
University referral center. PATIENTS: Fifty patients with cutaneous 
lymphoproliferative diseases. MAIN OUTCOME MEASURES: Proliferation indexes 
and apoptosis index calculated by using immunohistochemical techniques. 
RESULTS: The proliferation indexes in pseudolymphoma, which were calculated 
by using immunohistochemical analyses with anti-proliferating cell nuclear 
antigen and anti-MIB-1 monoclonal antibodies, were significantly lower than 
the indexes of MF, CTCL, BCL, and LyP, whereas, the apoptosis index in Lyp 
was significantly higher than in any other lymphoproliferative diseases 
studied. The apoptosis-proliferation ratio in the tumor stage of MF, CTCL, 
and BCL was almost constant, but the ratios in LyP and the plaque stage of 
MF were significantly higher than in the other diseases studied. 
CONCLUSIONS: The clinical behavior of each lymphoproliferative disease in 
the skin seemed to be reflected in the apoptosis and proliferation indexes. 
We conclude that these indexes may become useful factors in the 
determination of the diagnosis and the prognosis for patients with 
lymphoproliferative diseases. 

============================================================ 
22.) No evidence of HTLV-I proviral integration in lymphoproliferative 
disorders associated with cutaneous T-cell lymphoma. 
============================================================ 
Author 
Wood GS; Schaffer JM; Boni R; Dummer R; Burg G; Takeshita M; Kikuchi M 
Address 
Department of Dermatology, Case Western Reserve University, Cleveland, 
Ohio, USA. 
Source 
Am J Pathol, 150(2):667-73 1997 Feb 
Abstract 
Several recent studies have reported detection of HTLV-I genetic sequences 
in patients with cutaneous T-cell lymphoma (CTCL) including mycosis 
fungoides and Sezary syndrome. The purpose of this study was to determine 
whether HTLV-I was detectable in lesional tissues of patients suffering 
from diseases known to be associated with CTCL. Thirty-five cases were 
obtained from diverse geographical locations including Ohio, California, 
Switzerland, and Japan. Six of them had concurrent CTCL. Cases were 
analyzed using a combination of genomic polymerase chain reaction (PCR)/ 
Southern blot, dot blot, and Southern blot analyses. All assays were 
specific for HTLV-I provirus. Sensitivity ranged from approximately 10(-6) 
for PCR-based studies to 10(-2) for unamplified genomic blotting. Lesional 
DNA from patients with lymphomatoid papulosis (fourteen cases), Hodgkin's 
disease (twelve cases), and CD30+ large-cell lymphoma (nine cases) was 
tested for the HTLV-I proviral pX region using a genomic PCR assay followed 
by confirmatory Southern blot analysis with a nested oligonucleotide pX 
probe. All cases were uniformly negative. All of the Hodgkin's disease 
cases, eight of the large-cell lymphoma cases, and six of the lymphomatoid 
papulosis cases were then subjected to dot blot analysis of genomic DNA 
using a full-length HTLV-I proviral DNA probe that spans all regions of the 
HTLV-I genome. Again, all cases were negative. Finally, eleven of the 
Hodgkin's disease cases were also subjected to Southern blot analysis of 
EcoRI-digested genomic DNA using the same full-length HTLV-I probe. Once 
again, all cases were negative. These findings indicated that, despite 
utilization of a variety of sensitive and specific molecular biological 
methods, HTLV-I genetic sequences were not detectable in patients with 
CTCL-associated lymphoproliferative disorders. These results strongly 
suggest that the HTLV-I retrovirus is not involved in the pathogenesis of 
these diseases. 

============================================================ 
23.) Interleukin-7 receptor expression in cutaneous T-cell lymphomas. 
============================================================ 
Author 
Bagot M; Charue D; Boulland ML; Gaulard P; Revuz J; Schmitt C; Wechsler J 
Address 
Department of Dermatology, University Paris XII, H^opital Henri Mondor, 
Cr´eteil, France. 
Source 
Br J Dermatol, 135(4):572-5 1996 Oct 
Abstract 
Keratinocyte-derived interleukin-7 (IL-) is a potent growth factor for some 
cutaneous T-cell lymphomas (CTCL). We investigated the expression of IL-7 
receptor (IL-7R) in several types of cutaneous and nodal lymphomas. We 
studied 44 CTCL (13 mycosis fungoides, six S´ezary syndromes, eight 
pleomorphic small cell, and 17 pleomorphic medium and large cell), 10 
lymphomatoid papulosis (LP), five cutaneous B-cell lymphomas, and five 
reactive lymphocytic infiltrates. Twenty nodal T-cell lymphomas, and three 
reactive lymph nodes were also analysed. Frozen sections were stained with 
monoclonal antibodies directed against IL-7R, CD25, CD30 and T antigens 
(CD3, CD2, CD5, CD7, CD4, CD8), using the alkaline phosphatase-antialkaline 
phosphatase technique. No expression of IL-7R was observed in cutaneous 
B-cell lymphomas, benign cutaneous lymphoid infiltrates, and reactive lymph 
nodes. IL-7R was expressed by more than 20% of lymphoid cells in 50-75% of 
all histological subtypes of CTCL, and by more than 50% of cells in 15-50%. 
IL-7R was expressed by more than 20% and 50% of cells in 40% and 10% of 
nodal large T-cell lymphomas, respectively. Eighty-nine per cent of CTCL 
and LP expressing IL7-R also expressed CD25+, compared with 58% of 
IL-7R--CTCL and LP (P < 0.05). No association of IL7-R and CD30 expression 
was found. In conclusion, CTCL frequently express IL-7R. This expression is 
not related to the epidermotropic characteristic of the infiltrate. In CTCL 
and LP, IL-7R expression is associated to CD25 expression, but not to CD30 
expression. 

============================================================ 
24.) The t(2;5) in human lymphomas. 
============================================================ 
Author 
Kadin ME; Morris SW 
Address 
Department of Pathology, Beth Israel Hospital and Harvard Medical School, 
Boston, MA 02115, USA. [email protected] 
Source 
Leuk Lymphoma, 29(3-4):249-56 1998 Apr 
Abstract 
A recurrent, reciprocal balanced translocation, t(2;5) (p23;q35), has been 
recognized in CD30+ anaplastic large-cell lymphomas (ALCL), a newly 
recognized subtype comprising approximately 5% of all non-Hodgkin's 
lymphoma (NHL). This translocation creates a novel fusion protein, NPM-ALK, 
which has transforming properties in vitro and can cause large-cell 
lymphoma in vivo when transfected into murine bone marrow. Multiple 
techniques including reverse transcriptase-polymerase chain reaction 
(RT-PCR) amplification of NPM-ALK fusion transcripts, genomic DNA-PCR, RNA 
in situ hybridization, and fluorescence in situ hybridization (FISH) of 
metaphase chromosomes and interphase nuclei, and immunohistochemical 
detection of the 80 kilodalton protein (p80) derived from the NPM-ALK 
fusion have enabled surveys of normal and lymphoma tissues for evidence of 
the translocation. These studies suggest that expression of ALK protein, a 
novel orphan receptor tyrosine kinase, is normally confined to the nervous 
system. In lymphoma, NPM-ALK expression is most often seen in young 
patients with the monomorphic or small-cell variant of ALCL who present 
with advanced stage disease and have tumors with a CD30+, T- or null-cell 
phenotype. It is less frequently detected in older patients and in ALCL of 
pleomorphic histology. In addition, expression of NPM-ALK has been found in 
occasional CD30 negative B-cell lymphomas with diffuse large cell or 
immunoblastic histology. NPM-ALK is rarely, if ever, detected in Hodgkin's 
disease or secondary ALCL. Although initially found in primary nodal ALCL, 
recent studies suggest that NPM-ALK expression may occur in lymphoma at 
extranodal sites, including the skin; it remains controversial, however, 
whether CD30+ primary cutaneous lymphoma and its benign counterpart, 
lymphomatoid papulosis (LyP), express NPM-ALK in some cases. A 
retrospective study has suggested that expression of NPM-ALK is associated 
with a better overall 5-year survival; these results must be confirmed in 
prospective studies of patients with uniform staging and therapy to more 
fully understand the clinical significance of the t(2;5) and its novel 
chimeric protein, NPM-ALK. 

============================================================ 
25.) Pityriasis lichenoides in children: clinicopathologic review of 22 
patients. 
============================================================ 
Author 
Roman´i J; Puig L; Fern´andez-Figueras MT; de Moragas JM 
Address 
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, 
Spain. 
Source 
Pediatr Dermatol, 15(1):1-6 1998 Jan-Feb 
Abstract 
Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with 
an autoinvolutive course, that can occur in pediatric patients. 
Traditionally, acute and chronic variants have been described, but other 
special forms of presentation have been reported. We reviewed the clinical 
records and histopathologic specimens of all pediatric patients diagnosed 
with PL in our hospital from 1980 to 1995 to assess the clinicopathologic 
features of this disorder in our environment. Twenty-two of the 118 cases 
reviewed were pediatric patients less than 15 years old (12 males and 10 
females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with 
a mean of 9.3 years. Most of the patients (72%) had the chronic variant of 
the disease, while the remainder had an acute course. One patient suffered 
from acute ulceronecrotic PL. Systemic treatments prescribed were 
erythromycin in eight patients, PUVA in five patients, and methotrexate in 
one patient. Three patients had a prolonged course with more than two 
episodes. Acute and chronic PL are polar extremes, but individual cases 
cannot be classified only on the basis of histopathologic data, since 
coexistence of lesions in different stages of evolution can lead to 
sampling bias. Acute ulceronecrotic forms and the presence of a variable 
degree of cellular atypia in the infiltrate are liable to cause 
differential diagnostic problems with lymphomatoid papulosis (LP), which 
cannot be completely resolved on the basis of T-cell receptor clonal 
rearrangement detection. 

============================================================ 
Br J Dermatol 1999 Dec;141(6):1125-1128 
26.) Regional lymphomatoid papulosis: a report of four cases. 
============================================================ 
Scarisbrick JJ, Evans AV, Woolford AJ, Black MM, Russell-Jones R 
 

Lymphomatoid papulosis (LyP) is a chronic self-healing cutaneous eruption 
which is clinically benign but histologically malignant. Lesions occur 
episodically over the trunk and limbs. We describe four patients with 
regional LyP. All were male, with a range in age at onset from 12 to 47 
years. In all cases, lesions were confined to a segmental unilateral area. 
Two patients had type A and two type B LyP. We have long-term follow-up on 
one patient whose lesions were limited to the right buttock for more than 
20 years before more widespread lesions developed. Another patient with 
lesions on the left flank had mycosis fungoides limited to the same region. 
Only one other case of LyP presenting in a regional distribution has 
previously been described. 

============================================================ 
J Cutan Pathol 1999 Nov;26(10):520-2 
27.) Infection with parapoxvirus induces CD30-positive cutaneous 
infiltrates in humans. 
============================================================ 
Rose C, Starostik P, Brocker EB 
Department of Dermatology, University of Wurzburg, Germany. 

Expression of CD30 is a distinct feature of B- or T-cell activation, found 
in Hodgkin's disease, large cell anaplastic lymphoma, lymphomatoid 
papulosis, as well as in certain viral infections such as human 
T-lymphotropic virus type I, HIV, hepatitis B and C virus, and Epstein-Barr 
virus. Here, we report highly proliferative CD30-positive cutaneous 
infiltrates in 3 patients with Milkers's nodules, adding parapoxvirus 
infection to the spectrum of CD30-positive benign lympho-proliferations. 

============================================================ 
Blood 1999 Nov 1;94(9):3077-83 
28.) CD30-CD30 ligand interaction in primary cutaneous CD30(+) T-cell 
lymphomas: A clue to the pathophysiology of clinical regression. 
============================================================ 
Mori M, Manuelli C, Pimpinelli N, Mavilia C, Maggi E, Santucci M, Bianchi 
B, Cappugi P, Giannotti B, Kadin ME 
Department of Dermatology, University of Florence Medical School, Florence, 
Italy. 

Primary CD30(+) cutaneous T-cell lymphomas (CTCLs) represent a spectrum of 
non-Hodgkin's lymphomas (NHLs) that have been well defined at the clinical, 
histologic, and immunologic level. This group, which includes 2 main 
entities (large cell lymphoma and lymphomatoid papulosis [LyP]) and 
borderline cases, is characterized by the expression of CD30 antigen by 
neoplastic large cells at presentation, possible spontaneous regression of 
the skin lesions, and generally favorable clinical course. Although the 
functional relevance of CD30 and its natural ligand (CD30L) expression in 
most cases of NHL is presently undefined, previous studies indicate that 
CD30L is likely to mediate reduction of proliferation in CD30(+) anaplastic 
large-cell NHL. No information is currently available concerning the 
expression of CD30L in primary CD30(+) CTCLs. In this study, we 
investigated the immunophenotypic and genotypic expression of CD30 and 
CD30L in different developmental phases of skin lesions (growing v 
spontaneously regressing). By immunohistochemistry, CD30L expression was 
detected in regressing lesions only; by molecular analysis, the expression 
of CD30L was clearly higher in regressing lesions than in growing ones. 
CD30L, while expressed by some small lymphocytes, was most often 
coexpressed by CD30(+) neoplastic large cells, as demonstrated by 2-color 
immunofluorescence and by immunohistochemistry on paraffin sections. Taken 
together, these data suggest that CD30-CD30L interaction may play a role in 
the pathobiology of primary cutaneous CD30(+) lymphoproliferative 
disorders. In particular, CD30L (over)expression might have a major role in 
the mechanism of self-regression of skin lesions, the most distinctive 
clinical feature of this cutaneous lymphoma subtype. 
 

============================================================ 
Cancer 1999 Oct 1;86(7):1240-5 
29.) Increased risk of lymphoid and nonlymphoid malignancies in patients 
with lymphomatoid papulosis. 
============================================================ 
Wang HH, Myers T, Lach LJ, Hsieh CC, Kadin ME 
Department of Pathology, Beth Israel Deaconess Medical Center and Harvard 
Medical School, Boston, Massachusetts 02115, USA. 

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare skin disease with 
malignant potential. The long term outcomes of patients with this disease 
have not been adequately assessed. METHODS: Fifty-seven patients with 
biopsy-proven LyP and 67 controls matched for age, gender, and race were 
followed prospectively from 1988 to 1996. Reported malignancies were 
confirmed by surgical pathology and/or autopsy reports. A search through 
the National Death Index through December 1995 was conducted to identify 
all deaths, and death certificates were procured. Expected numbers of 
malignancies based on SEER data were calculated for both the patient and 
the control groups. RESULTS: Six LyP patients (10.5%) and 1 control (1.5%) 
reported nonlymphoid malignancies (P = 0.047). Two patients and no controls 
developed lymphoid malignancies (mycosis fungoides and CD30(+) cutaneous 
lymphoma). The expected numbers of nonlymphoid and lymphoid malignancies in 
the LyP patient group, based on the SEER data, were 1.93 and 0.15, 
respectively, yielding a relative risk (with 95% confidence interval) of 
3.11 (1.26-6.47) for nonlymphoid malignancies and 13.33 (2.24-44.05) for 
malignant lymphomas in the LyP patients. There was no significant 
difference between the observed and expected numbers of malignancies in the 
control group. Four LyP patients died during the follow-up, three due to 
malignancies; and one control died of a gunshot wound to the head 
(suicide). The difference in overall survival between the LyP patients and 
the controls was not statistically significant (P = 0. 12). CONCLUSIONS: 
Patients with LyP appear to have an increased risk of both lymphoid and 
nonlymphoid malignancies. The increased risk of nonlymphoid as well as 
lymphoid malignancies may suggest a basic underlying genetic defect leading 
to the development of malignancy in LyP patients. Copyright 1999 American 
Cancer Society. 

============================================================ 
Am J Pathol 1999 Aug;155(2):483-92 
30.) Primary cutaneous CD8-positive epidermotropic cytotoxic T cell 
lymphomas. A distinct clinicopathological entity with an aggressive 
clinical behavior. 
============================================================ 
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R 
Institute of Dermatologic Science, IRCCS Ospedale Maggiore, Milan, Italy. 
[email protected] 

Cutaneous T cell lymphomas (CTCL) generally have the phenotype of CD3+, 
CD4+, CD45RO+ memory T cells. CTCL expressing a CD8+ T cell phenotype are 
extremely rare and ill-defined. To elucidate whether these CD8+ CTCL 
represent a distinct disease entity, the clinical, histological, and 
immunophenotypical features of 17 CD8+ CTCL were reviewed. None of the 17 
cases expressed markers characteristic of natural killer cells or 
gamma/delta T cells. Nine of 17 cases showed the characteristic clinical 
and histological features as well as clinical behavior of well defined 
types of CTCL, such as mycosis fungoides (2 cases), pagetoid reticulosis (2 
cases), lymphomatoid papulosis (2 cases), and CD30+ large T cell lymphoma 
(2 cases), all of which usually express a CD4+ T cell phenotype, and 1 case 
of subcutaneous panniculitis-like T cell lymphoma. The other 8 cases formed 
a homogeneous group showing a distinctive set of clinicopathological and 
immunophenotypical features, not consistent with that of other well defined 
types of CTCL. Clinical characteristics included presentation with 
generalized patches, plaques, papulonodules, and tumors mimicking 
disseminated pagetoid reticulosis; metastatic spread to unusual sites, such 
as the lung, testis, central nervous system, and oral cavity, but not to 
the lymph nodes; and an aggressive course (median survival, 32 months). 
Histologically, these lymphomas were characterized by band-like infiltrates 
consisting of pleomorphic T cells or immunoblasts, showing a diffuse 
infiltration of an acanthotic epidermis with variable degrees of 
spongiosis, intraepidermal blistering, and necrosis. The neoplastic cells 
showed a high Ki-67 proliferation index and expression of CD3, CD8, CD7, 
CD45RA, betaF1, and TIA-1 markers, whereas CD2 and CD5 were frequently 
lost. Expression of TIA-1 pointed out that these lymphomas are derived from 
a cytotoxic T cell subset. The results of this and other studies reviewed 
herein suggest that these strongly epidermotropic primary cutaneous CD8+ 
cytotoxic T cell lymphomas represent a distinct type of CTCL with an 
aggressive clinical behavior. 

============================================================ 
Br J Dermatol 1998 Oct;139(4):630-8 
31.) Lymphomatoid papulosis in association with mycosis fungoides: a study 
of 15 cases. 
============================================================ 
Basarab T, Fraser-Andrews EA, Orchard G, Whittaker S, Russel-Jones R 
St John's Institute of Dermatology, St Thomas' Hospital, London, UK. 

We report clinical findings in 15 patients with lymphomatoid papulosis 
(LyP) associated with mycosis fungoides (MF). LyP either preceded (n = 4), 
followed (n = 5) or occurred concurrently with the MF lesions (n = 6). 
Twenty-eight LyP lesions were classified histologically and analysed 
further with immunostaining for CD3 and CD30. Five biopsies contained a 
predominance of type A cells, six biopsies contained a predominance of type 
B cells. and six were mixed (A + B). However, 11 biopsies contained a 
population of atypical mononuclear cells with large hyperchromatic nuclei 
that we have termed indeterminate cells. These cells contained a thin rim 
of eosinophilic cytoplasm and showed strong CD30 but absent, faint or 
normal CD3 staining. In seven biopsies from five separate patients these 
cells represented the predominant cell type and we have termed this the 
pleomorphic variant of LyP. Analysis of T-cell receptor genes using 
Southern blot analysis and polymerase chain reaction/single strand 
conformational polymorphism analysis identified a T-cell clone in six of 16 
LyP lesions and nine of 16 MF lesions. In the three patients who had clones 
in both types of skin lesions, the clones were identical. Only two of 10 
blood samples, both of which were from the same patient, had a T-cell clone 
and none of two lymph nodes showed evidence of a clonal population. To date 
all patients are alive with a median follow-up of 15 years from the onset 
of the first lesion. One patient has developed Lin anaplastic large cell 
lymphoma of the nasopharynx. These data augment the current literature on 
the association of LyP and MF and suggest that the 

============================================================ 
Oncology (Huntingt) 1998 Oct;12(10):1521-30; discussion 1532-4 
32.) Early detection of cutaneous lymphoma. 
============================================================ 
Abd-el-Baki J, Stefanato CM, Koh HK, Demierre MF, Foss FM 
Department of Dermatology, Boston University School of Medicine, 
Massachusetts, USA. 

Cutaneous lymphomas comprise a spectrum of diseases characterized by 
infiltration of the skin by malignant lymphocytes. The clinical 
manifestations of cutaneous lymphomas vary, and they can mimic benign 
dermatoses, as well as nodal or visceral malignancies with cutaneous 
spread. Cutaneous lymphomas are divided into T-cell lymphomas and B-cell 
lymphomas. Cutaneous T-cell lymphomas include mycosis fungoides, Sezary 
syndrome, lymphomatoid papulosis, CD30+ large cell lymphoma, and adult 
T-cell leukemia/lymphoma. The extent and severity of skin manifestations in 
cutaneous T-cell lymphomas are prognostic indicators of extracutaneous 
involvement. Primary cutaneous B-cell lymphomas comprise 10% to 25% of all 
primary cutaneous non-Hodgkin's lymphomas and are classified according to 
their cell of origin. Most cutaneous B-cell lymphomas have an indolent 
course and excellent prognosis when compared to their nodal counterparts. 
Many factors have been implicated in the etiology of cutaneous lymphomas, 
including chemical and drug exposures, as well as microbial agents, such as 
the Epstein-Barr virus (EBV), human T-lymphocyte virus-1 (HTLV-1), and 
Borrelia burgdorferi. Immunohistochemistry and lymphocyte-receptor gene 
rearrangement studies are useful in distinguishing malignant from benign 
conditions. 

============================================================ 
J Am Acad Dermatol 1998 Jun;38(6 Pt 1):877-95; quiz 896-7 
33.) Cutaneous pseudolymphomas. 
============================================================ 
Ploysangam T, Breneman DL, Mutasim DF 
Department of Dermatology, University of Cincinnati Medical Center, Ohio, 
USA. 

Cutaneous pseudolymphoma refers to a heterogeneous group of benign reactive 
T- or B-cell lymphoproliferative processes of diverse causes that simulate 
cutaneous lymphomas clinically and/or histologically. The inflammatory 
infiltrate is bandlike, nodular, or diffuse and is composed predominantly 
of lymphocytes with or without other inflammatory cells. Depending on the 
predominant cell type in the infiltrate, cutaneous pseudolymphomas are 
divided into T- and B-cell pseudolymphomas. Cutaneous T-cell 
pseudolymphomas include idiopathic cutaneous T-cell pseudolymphoma, 
lymphomatoid drug reactions, lymphomatoid contact dermatitis, persistent 
nodular arthropod-bite reactions, nodular scabies, actinic reticuloid, and 
lymphomatoid papulosis. Cutaneous B-cell pseudolymphomas include idiopathic 
lymphocytoma cutis, borrelial lymphocytoma cutis, tattoo-induced 
lymphocytoma cutis, post-zoster scar lymphocytoma cutis, and some 
persistent nodular arthropod-bite reactions. This review attempts to 
discuss current aspects of the classification, pathogenesis, clinical 
spectrum, histopathologic and immunohistochemical diagnosis, and laboratory 
investigations for clonality in the various types of cutaneous 
pseudolymphomas. 
 

============================================================ 
Pediatr Dermatol 1998 Mar-Apr;15(2):146-7 
34.) Lymphomatoid papulosis in childhood with exclusive acral involvement. 
============================================================ 

Thomas GJ, Conejo-Mir JS, Ruiz AP, Linares Barrios M, Navarrete M 
Publication Types: 

Letter 
============================================================ 

============================================================ 
J Invest Dermatol 1997 Dec;109(6):817-8 
35.) Absence of HTLV-1 proviral sequences in patients with lymphomatoid 
papulosis. 
============================================================ 
Ortiz Romero PL, Vallejo A, Lopez Estebaranz JL, Garcia Saiz A, Fernandez 
V, Iglesias Diez L 
Publication Types: 

letter 
============================================================ 

============================================================ 
Arch Dermatol 1997 Sep;133(9):1081-6 
36.) Clinicopathologic manifestations of Epstein-Barr virus-associated 
cutaneous lymphoproliferative disorders. 
============================================================ 
Iwatsuki K, Ohtsuka M, Harada H, Han G, Kaneko F 
Department of Dermatology, Fukushima Medical College, Japan. 

OBJECTIVE: To elucidate clinicopathologic manifestations of cutaneous 
lymphoproliferative disorders associated with Epstein-Barr virus (EBV) 
infection. DESIGN: Retrospective survey of case series. SETTING: University 
hospital medical center. PATIENTS: Sixty-five patients with cutaneous 
lymphomas and related disorders. MAIN OUTCOME MEASURES: Detection of EBV 
genes and EBV-encoded small nuclear RNAs. RESULTS: Evidence of latent EBV 
infection was demonstrated in 15 patients: 3 had malignant lymphoma with 
clinical features mimicking cytophagic histiocytic panniculitis, 6 had 
facial vesiculopapular eruptions mimicking hydroa vacciniforme, 4 had 
angiocentric lymphoma, 1 had histiocytoid lymphoma associated with 
hemophagocytosis, and 1 had plasmacytoma. Hypersensitivity to mosquito 
bites was noted in a patient with hydroa vacciniforme-like eruptions and 
another with histiocytoid lymphoma. Angiocentric infiltration of atypical 
lymphoid cells was a common histological feature in the patients with 
hydroa vacciniforme-like eruptions and angiocentric lymphoma. No evidence 
of EBV infection was apparent in 19 patients with mycosis fungoides or 
Sezary syndrome, 7 with adult T-cell leukemia or lymphoma, 3 with 
lymphomatoid papulosis (type A), and 2 with lymphocytoma cutis. CONCLUSION: 
Patients with EBV-associated cutaneous lymphoproliferative disorders 
present with unique and diagnostic clinicopathologic features distinct from 
those of mycosis fungoides or Sezary syndrome. 

============================================================ 
Acta Derm Venereol 1997 Sep;77(5):403 
37.) Follicular lymphomatoid papulosis and multiple myeloma. 
============================================================ 

Rongioletti F, Basso GI, Sementa A, Gambini C, Rebora A 
Publication Types: 

Letter 
============================================================ 
============================================================ 
38.) Lymphomatoid papulosis--treatment with recombinant interferon alfa-2a
and 
etretinate. 
============================================================ 
SO - Dermatology 1995;190(4):288-91 
AU - Wyss M; Dummer R; Dommann SN; Joller-Jemelka HI; Dours-Zimmermann MT; 
Gilliet F; Burg G 
PT - JOURNAL ARTICLE 
AB - Lymphomatoid papulosis is a rare cutaneous lymphoproliferative
disorder with 
nodular, papulonecrotic or plaque-like lesions. Although it is clinically
benign, the 
histology shows large, atypical lymphoid cells that display antigenic
markers of activated 
T-helper lymphocytes and express CD30. There is a close relationship to
Hodgkin's 
disease and to Ki-1-positive anaplastic large-cell lymphoma of the skin.
For therapy, 
various modalities such as PUVA, steroids and acyclovir have been used. We
report on a 
patient with a 10-year history of disease. Treatment with interferon
alfa-2a, 3 MU 3 
times/week for 4 weeks, and etretinate, 50 mg/day for 5 months, was
initially successful, 
but lesions further relapsed 5 months after cessation of the therapy. 

============================================================ 
39.) Lymphomatoid papulosis: a follow-up study of 41 patients. 
============================================================ 
SO - Semin Dermatol 1994 Sep;13(3):197-201 
AU - Christensen HK; Thomsen K; Vejlsgaard GL 
PT - JOURNAL ARTICLE 
AB - Forty-one patients with lymphomatoid papulosis have been followed
from 1 to 22 
years (mean 11.4 years, median 10 years). Six patients developed malignant
lymphoma, 3 
cutaneous T-cell lymphoma, 2 Ki-1 large cell lymphoma, and 1 Hodgkin's
disease. A 
clinical malignant presentation combined with the finding of aneuploidy in
skin lesions 
seem to be indications of a malignant potential. Treatment with
methotrexate in low 
dosage is an efficient treatment of lymphomatoid papulosis and probably
diminishes the 
risk of malignancy. 

============================================================ 
40.) Lymphomatoid papulosis associated with acquired ichthyosis. 
============================================================ 
SO - J Am Acad Dermatol 1994 May;30(5 Pt 2):889-92 
AU - Yokote R; Iwatsuki K; Hashizume H; Takigawa M 
PT - JOURNAL ARTICLE 
AB - We describe a 64-year-old man with lymphomatoid papulosis associated
with 
acquired ichthyosis. The papulonodular lesions were composed of large
atypical 
lymphocytes positive for CD3, CD4, and Ki-1. The ichthyosiform eruption
also occurred 
on the extremities and had the histologic features of ichthyosis vulgaris.
Although 
monoclonality of infiltrating cells could not be demonstrated, acquired
ichthyosis appears 
to be induced in patients with lymphomatoid papulosis by the same
pathomechanism 
underlying other lymphoproliferative diseases. 

============================================================ 
41.) Primary anaplastic large-cell lymphoma of the skin. A case report
suggesting that 
regressing atypical histiocytosis and lymphomatoid papulosis are subsets. 
============================================================ 
SO - J Am Acad Dermatol 1994 Feb;30(2 Pt 2):358-63 
AU - Yashiro N; Kitajima J; Kobayashi H; Fushida H; Nakagawa K; Furukawa M; 
Hamada T 
PT - JOURNAL ARTICLE 
AB - A patient with primary anaplastic large-cell lymphoma of the skin
with characteristic 
clinical findings is described. The diagnosis was made on the basis of
histologic and 
immunohistochemical findings. The phenotype of the tumor cells was not
determined, but 
rearrangement of the T-cell receptor beta gene indicated that the tumor was
of T-cell 
lineage. Despite high-grade malignancy of the tumor cells, the patient
unexpectedly had a 
benign clinical course. The findings in this case suggest that regressing
atypical 
histiocytosis and lymphomatoid papulosis type A are subsets of anaplastic
large-cell 
lymphoma. 

============================================================ 
42.) Lymphomatoid papulosis: a clinical and histopathologic review of 53
cases with 
leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene 
rearrangement studies. 
============================================================ 
SO - J Am Acad Dermatol 1994 Feb;30(2 Pt 1):210-8 
AU - el-Azhary RA; Gibson LE; Kurtin PJ; Pittelkow MR; Muller SA 
PT - JOURNAL ARTICLE 
AB - BACKGROUND: Lymphomatoid papulosis (LyP) is a recurrent hemorrhagic 
papular skin eruption with a clinically benign course and histopathologic
features of 
lymphoma. OBJECTIVE AND METHODS: To better characterize this disease, we 
studied 53 patients seen since 1965. RESULTS: A lymphoproliferative
malignancy 
developed within 2 to 36 years after onset of LyP in eight patients.
Histologically, the 
dermis in LyP showed an infiltrate of large (type A) or small (type B)
atypical 
lymphocytes. The large atypical cells (type A) stained with CD30 (Ber-H2).
Seven of the 
patients in whom lymphoma developed had type A histologic features. DNA flow 
cytometry showed mainly a diploid pattern, except for two cases that showed
aneuploidy. 
Five of 11 patients showed T-cell receptor (TCR) clonal gene
rearrangements; lymphoma 
has not developed in these patients. One patient had a TCR rearrangement in
a plaque of 
mycosis fungoides but not in the LyP lesion. CONCLUSION: LyP is either a
reactive 
skin condition or a localized lymphoid malignancy. Neither DNA flow
cytometry nor TCR 
gene rearrangement can predict the 15% to 19% of patients in whom a
lymphoma will 
develop. Continued observation of all patients is essential. 

============================================================ 
43.) [Lymphomatoid papulosis and anaplastic giant-cell lymphoma] 
============================================================ 
SO - Ann Dermatol Venereol 1994;121(10):727-30 
AU - Moreau-Cabarrot A; Bonafe JL; Gorguet B; Andrieu H; Massip P 
PT - JOURNAL ARTICLE 
AB - INTRODUCTION. The association between lymphomatoid papulosis and 
malignant Hodgkin or non-Hodgkin lymphoma is well known but still raises
the problem 
of nosology between these two pathologies. Is lymphomatoid papulosis a 
pseudolymphoma, a prelymphomatous state or a true skin lymphoma? CASE REPORT. 
We observed a patient who had lymphomatoid papulosis and anaplastic
large-cell 
lymphoma within an interval of 8 years between. This case was particularly
interesting 
because identical immunophenotypes were observed in the atypical
large-cells of the skin 
and the lymphomatous cells of the lymph nodes (positive for CD43, CD45, CD25, 
CD30, CD15, EMA). DISCUSSION. This case points out that atypical
large-cells of 
lymphomatoid papulosis express the CD15 antigen which is only expressed by
atypical 
large-cells in half of the cases of lymphomatoid papulosis. In addition,
EMA is classically 
expressed in primary lymph node lymphomas rather than in primary cutaneous
anaplastic 
large cell lymphomas which could predict extracutaneous dissemination of
lymphomatoid 
papulosis. Furthermore, the demonstration that the skin lesions and the
lymph nodes 
responded differently to the same treatment would suggest that there are
other 
unrecognized biological differences. Lymphomatoid papulosis appears to be a
range of 
disorders of the lymphoproliferation of activated T-cells and could include
varioliform 
parapsoriasis and cutaneous lymphoma. 

============================================================ 
44.) Molecular evidence for a clonal relationship between lymphomatoid
papulosis and 
Ki-1 positive large cell anaplastic lymphoma. 
============================================================ 
SO - J Dermatol Sci 1993 Oct;6(2):121-6 
AU - Volkenandt M; Bertino JR; Shenoy BV; Koch OM; Kadin ME 
PT - JOURNAL ARTICLE 
AB - Currently, considerable controversy surrounds questions about the
clonal evolution 
of lymphomas in patients with lymphomatoid papulosis. In order to analyze a
possible 
clonal relationship between lesions of lymphomatoid papulosis and a Ki 1+
large cell 
anaplastic lymphoma in the same patient, a highly specific molecular probe
for the 
malignant lymphoid clone of the large cell anaplastic lymphoma was
developed. As a 
clone specific molecular marker, highly variable junctional sequences of
rearranged T-cell 
receptor-gamma genes were used. An oligonucleotide primer complementary to
these 
sequences was synthesized and, using the polymerase chain reaction, clone
specific DNA 
was detected in all lesions of lymphomatoid papulosis of the patient. These
results provide 
evidence for a clonal relationship between lesions of lymphomatoid
papulosis and large 
cell anaplastic lymphoma developing in the same patient. 

============================================================ 
45.) Lymphomatoid papulosis followed by Hodgkin's lymphoma. Differential
response to 
therapy [see comments] 
============================================================ 
SO - Arch Dermatol 1993 Jan;129(1):86-91 
AU - Zackheim HS; Le Boit PE; Gordon BI; Glassberg AB 
PT - JOURNAL ARTICLE; REVIEW (32 references); REVIEW OF REPORTED 
CASES 
AB - BACKGROUND. The association of lymphomatoid papulosis (LyP) with 
Hodgkin's lymphoma or other lymphomas is well recognized. However, the
issue as to 
whether this represents an independent association or a transformation of
one proliferative 
process to the other remains unresolved. OBSERVATION. A woman with LyP 
subsequently developed Hodgkin's lymphoma. Combination chemotherapy
resulted in 
apparent cure of the lymphoma but had only a transient effect on the LyP. A
literature 
review revealed a similar difference in response to chemotherapy or
radiation therapy in 
most patients who had LyP and associated lymphoma. CONCLUSIONS. The 
differential response to therapy in patients with LyP and associated
lymphoma suggests 
that there are biological differences between LyP cells and associated
lymphoma cells 
even though in some patients the immunophenotype and genotype were reported
to be 
identical. However, alternative explanations are possible. In this article
we also review 
studies on other cases of LyP associated with Hodgkin's lymphoma. 

============================================================ 
46.) Epidemiology of lymphomatoid papulosis [see comments] 
============================================================ 
SO - Cancer 1992 Dec 15;70(12):2951-7 
AU - Wang HH; Lach L; Kadin ME 
PT - JOURNAL ARTICLE 
AB - BACKGROUND. Lymphomatoid papulosis is a rare skin disease with
malignant 
potential. Its epidemiology is largely unknown. METHODS. A case-control
study of 
lymphomatoid papulosis was done to characterize the patient population and
investigate 
the risk factors for its development. Fifty-seven patients with biopsy-proven 
lymphomatoid papulosis and 67 individually matched control subjects who
were recruited 
among relatives and acquaintances of the patients answered a standard
questionnaire over 
the telephone. RESULTS. Among patients with lymphomatoid papulosis, 3 had a
history 
of Hodgkin disease, 3 had non-Hodgkin lymphoma, and 10 had mycosis
fungoides; none 
of the control subjects reported such histories. No significant differences
were observed 
between patients and control subjects in regard to residence or travel
history or 
exposures to various physical, chemical, and biologic agents. A higher,
although not 
statistically significant, percentage of patients than control subjects
reported a history of 
radiation therapy and nonlymphoid malignant lesions. No differences were
found between 
patients and control subjects in regard to other medical conditions or
family medical 
history. CONCLUSIONS. Patients with lymphomatoid papulosis have a
significantly 
increased frequency of prior or coexisting lymphoproliferative disorders,
an increased 
frequency of nonlymphoid malignant lesions, and exposure to radiation
therapy. 

============================================================ 
47.) Lethal midline granuloma (peripheral T-cell lymphoma) after lymphomatoid 
papulosis. 
============================================================ 
SO - Cancer 1992 Aug 15;70(4):835-9 
AU - Harabuchi Y; Kataura A; Kobayashi K; Yamamoto T; Yamanaka N; Hirao M; 
Onodera K; Kon S 
PT - JOURNAL ARTICLE 
AB - A Japanese woman with an 8-year history of lymphomatoid papulosis
(LP) had 
lethal midline granuloma (LMG) develop at the age of 51 years. There were
histologic 
similarities between LP and LMG seen in this patient. Surface phenotypic
studies on nasal 
and cutaneous lesions demonstrated a population of T-cells expressing CD2,
CD4, 
CD25, CD30, and histocompatibility antigen-DR (HLA-DR). Genotypic analyses of 
nasal and skin biopsy specimens disclosed a clonal rearrangement of the
beta T-cell 
receptor gene with the same rearrangement pattern. These data indicate that
this patient 
had LMG characterized by clonal peripheral T-cell lymphoma, which probably
resulted 
from progression of the LP. 

============================================================ 
48.) The prognosis of patients with lymphomatoid papulosis associated with
malignant 
lymphomas. 
============================================================ 
SO - Br J Dermatol 1992 Jun;126(6):596-602 
AU - Beljaards RC; Willemze R 
PT - JOURNAL ARTICLE; REVIEW (38 references); REVIEW OF REPORTED 
CASES 
AB - Lymphomatoid papulosis (LyP) is a disorder which generally runs a
benign course, 
but can sometimes be associated with a malignant lymphoma. Information
about the 
prognosis of these LyP-associated lymphomas is, however, fragmentary. In
this study, the 
clinical data of 50 LyP-associated malignant lymphomas, including 11
patients of our own 
group and 39 reported in the literature, are evaluated. Three main groups of 
LyP-associated malignant lymphomas could be distinguished: cases associated
with 
mycosis fungoides (19/50 cases). Hodgkin's disease (12/50 cases) and (CD30+) 
large-cell lymphomas (16/50). The results of this study demonstrate that
patients with 
mycosis fungoides. Hodgkin's disease, and (CD30+) large-cell lymphomas
limited to the 
skin have a favourable prognosis. However, the prognosis of patients
developing a 
systemic (CD30+) large-cell lymphoma proved generally poor. The results of
this study 
also indicate that the risk of an individual LyP patient developing
systemic lymphoma is 
less than 5%. 

============================================================ 
49.) Immunophenotypic and genotypic characterization of lymphomatoid
papulosis. 
============================================================ 
SO - J Am Acad Dermatol 1992 Jun;26(6):968-75 
AU - Parks JD; Synovec MS; Masih AS; Braddock SW; Nakamine H; Sanger WG; 
Harrington DS; Weisenburger DD 
PT - JOURNAL ARTICLE; REVIEW (24 references); REVIEW, TUTORIAL 
AB - BACKGROUND: Lymphomatoid papulosis (LyP) is a chronic dermatosis that 
histologically resembles malignant lymphoma. Thus far, only a few cases of
LyP have 
been characterized in detail with regard to immunophenotype, genotype, and
karyotype. 
OBJECTIVE: Our purpose was to study seven patients with LyP and compare the
results 
to those reported in the literature. METHODS: Skin biopsy specimens were
analyzed by 
frozen section immunohistochemical and molecular biologic techniques.
Cytogenetic 
analysis was also performed in three cases. RESULTS: The atypical lymphoid
cells 
consisted of activated helper T cells; four of the seven patients had
lesions with a 
detectable clonal T-cell population. A peripheral T-cell lymphoma developed
in one 
patient before the emergence of a genotypically different LyP T-cell clone.
Cytogenetic 
studies were abnormal in one case of LyP and normal in another, whereas the
karyotype 
of the lymphoma was abnormal. CONCLUSION: LyP is a preneoplastic
proliferation of 
activated helper T cells, which is often clonal and may regress and expand
with the 
development of new LyP clones or lymphoma. 

============================================================ 
50.) Pityriasis lichenoides and lymphomatoid papulosis. 
============================================================ 
SO - Semin Dermatol 1992 Mar;11(1):73-9 
AU - Rogers M 
PT - JOURNAL ARTICLE; REVIEW (79 references); REVIEW, TUTORIAL 
AB - The clinical features, histopathology, immunopathology, and
management of 
pityriasis lichenoides and lymphomatoid papulosis are discussed, with
particular emphasis 
on the pediatric aspects of these conditions. The difficulties in logically
separating pityriasis 
lichenoides into an acute (pityriasis lichenoides et varioliformis acuta)
and a chronic 
(pityriasis lichenoides chronical) form are addressed. The development of
lymphoreticular 
malignancy in patients with lymphomatoid papulosis has been well
documented, but 
pityriasis lichenoides has characteristically been regarded as a benign
condition. However, 
recent reports of the development of large plaque parapsoriasis in patients
with pityriasis 
lichenoides have led to a reconsideration. Some of these patients were in
the pediatric age 
group. Although there are significant clinical, histopathological, and
immunopathological 
differences between pityriasis lichenoides and lymphomatoid papulosis, the
demonstration 
of similar clonal T cell receptor gene rearrangements and the confirmation
of the 
potentially premalignant nature of both suggests that there may indeed be an 
interrelationship between these two controversial entities. Close follow-up
of patients with 
both of these conditions is recommended, with observation being
discontinued only when 
the patient has been free of lesions for several years. 

============================================================ 
51.) Lymphomatoid papulosis: clinicopathological comparative study with
pityriasis 
lichenoides et varioliformis acuta. 
============================================================ 
SO - J Dermatol 1991 Oct;18(10):580-5 
AU - Erpaiboon P; Mihara I; Niimura M 
PT - JOURNAL ARTICLE 
AB - We have compared the clinical and histopathological features of 6
patients with 
lymphomatoid papulosis (LP) and 14 patients with pityriasis lichenoides et
varioliformis 
acuta (PLEVA). There were some differences between the clinical features in
the two 
diseases, including the size and appearance of skin lesions and the
duration of the course 
of disease. Ki-1 Ag positive, large, atypical, lymphoid cells were always
seen in 
lymphomatoid papulosis; none of lymphoid cells of pityriasis lichenoides et
varioliformis 
acuta demonstrated this antigen. We conclude that lymphomatoid papulosis
and PLEVA, 
although sharing some common features, should be considered to be different
clinical and 
immunopathological entities. 

============================================================ 
52.) PUVA-induced lymphomatoid papulosis in a patient with mycosis fungoides. 
============================================================ 
SO - J Am Acad Dermatol 1991 Aug;25(2 Pt 2):422-6 
AU - Wolf P; Cerroni L; Smolle J; Kerl H 
PT - JOURNAL ARTICLE 
AB - The occurrence of lymphomatoid papulosis in patients with cutaneous
lymphoma, 
particularly mycosis fungoides, has been described in medical literature. A
68-year-old 
woman affected by mycosis fungoides in the plaque stage noticed that multiple 
papulonodular lesions of lymphomatoid papulosis developed suddenly after a
few 
sessions of PUVA therapy. The PUVA induction of lymphomatoid papulosis was 
confirmed by the appearance of new lesions after a second cycle of PUVA
exposure on a 
limited area of the body. Complete regression of all PUVA-induced
lymphomatoid 
papulosis lesions was achieved within a few weeks with oral prednisone and
topical 
steroids. During the entire treatment the patches and plaques of mycosis
fungoides 
persisted unchanged. 

============================================================ 
53.) Accuracy in diagnosis of lymphomatoid papulosis. 
============================================================ 
SO - Am J Dermatopathol 1991 Feb;13(1):20-5 
AU - Cockerell CJ; Stetler LD 
PT - JOURNAL ARTICLE 
AB - This study was undertaken to assess the accuracy of histologic
diagnosis of 
lymphomatoid papulosis (LyP), which may be confused with malignant lymphoma
or 
other entities. It is essential that accurate diagnoses be made because LyP
may be a 
marker for malignant lymphoma. All 15 examples of LyP reviewed in a
dermatopathology 
laboratory during a 14-year period and 180 histologic sections of tissue
that could be 
confused with LyP were reviewed. Criteria for diagnosis of LyP were applied
without 
benefit of clinical history, and revised diagnoses were made where
indicated. Clinical 
follow-up information was obtained and original accuracy of diagnosis was
assessed by 
comparing clinical courses with original histologic diagnoses. In cases of
LyP in which 
numerous atypical lymphoid cells were present, 100% accuracy was noted.
When fewer 
atypical lymphoid cells were present and inflammatory cell infiltrates were
less dense, the 
diagnosis was less certain. Overall, a 64% correlation of clinical course
and histologic 
diagnosis of LyP was noted. We conclude that the histologic diagnosis of
LyP is generally 
reliable and accurate; however, in some cases a precise diagnosis cannot be
made with 
certainty. Cases with fewer atypical lymphoid cells may fail to correlate
well with the 
classic course of LyP and may represent a variant or histologic simulator. 

============================================================ 
54.)Regressing atypical histiocytosis and lymphomatoid papulosis: variants
of the same 
disorder? 
============================================================ 
SO - Br J Dermatol 1990 Oct;123(4):515-21 
AU - Cerio R; Black MM 
PT - JOURNAL ARTICLE 
AB - We report a patient with lymphomatoid papulosis who developed a
lesion with the 
clinicopathological features of regressing atypical histiocytosis.
Immunohistochemical 
studies supported a T-cell histogenesis and many of the atypical cells
demonstrated 
BerH2 (Ki-I antigen) positivity. The case supports the view that regressing
atypical 
histiocytosis and lymphomatoid papulosis are different manifestations of
the same disease 
spectrum. 

============================================================ 
55.) Lymphomatoid papulosis and its relationship to "idiopathic"
hypereosinophilic 
syndrome. 
============================================================ 
SO - J Am Acad Dermatol 1988 Feb;18(2 Pt 1):339-44 
AU - Whittaker SJ; Jones RR; Spry CJ 
PT - JOURNAL ARTICLE 
AB - Persistent hypereosinophilia, endomyocardial fibrosis, and a
recurrent self-healing 
papulonodular eruption with the histologic features of lymphomatoid
papulosis are 
described in three patients. One patient died after developing an acute
myeloblastic 
transformation in the eosinophil series. Immunocytochemical studies of
cutaneous lesions 
in two of the patients suggested a mature T-cell phenotype with a
predominant population 
of CD4-positive cells. Immunostaining of cutaneous tissue with monoclonal
antibodies 
BE1 and BE2 yielded negative findings. Because it is now known from in
vitro studies that 
T lymphocytes secrete the eosinopoietic factor, interleukin 5, it is
possible that the 
cutaneous lesions, hypereosinophilia, and associated endomyocardial
fibrosis were 
induced by transformed helper T lymphocytes in these three patients. 

============================================================ 
56.) Lymphomatoid papulosis/pityriasis lichenoides in two children. 
============================================================ 
SO - Pediatr Dermatol 1987 Nov;4(3):238-41 
AU - Ashworth J; Paterson WD; MacKie RM 
PT - JOURNAL ARTICLE 
AB - Two children developed lymphomatoid papulosis/pityriasis lichenoides
at ages 3 
and 6 years. Follow-up continued for 13 years in the former patient and for
6 years in the 
latter. Both children now have continuing low-grade disease activity
requiring in the one 
case topical corticosteroid therapy and in the other low-dose systemic
steroid therapy. 
These children are reported to emphasize to pediatricians, pediatric
pathologists, and 
hematologists that pseudolymphomatous conditions can exist in young
children and do not 
require potent cytotoxic therapy. In both of our patients, the initial
diagnosis was thought 
to be an aggressive lymphoma. 

============================================================ 
57.) [Acyclovir in mycosis fungoides and lymphomatoid papulosis] 
============================================================ 
SO - Hautarzt 1986 Oct;37(10):533-6 
AU - Burg G; Klepzig K; Kaudewitz P; Wolff H; Braun-Falco O 
PT - CLINICAL TRIAL; JOURNAL ARTICLE 
AB - A survey is given on 23 patients (10 of our own, 13 reported in
personal 
communications and in the literature) suffering from lymphoproliferative
diseases and 
treated with acyclovir (ACV). In 5 patients (3 of 18 with cutaneous T-cell
lymphomas, 2 
of 5 with lymphomatoid papulosis) partial remission could be achieved.
Since herpes 
simplex virus, cytomegalovirus and viruses like Epstein-Barr and
varicella-zoster do not 
play an etiologic role and since HTLV-I virus, due to its lack of thymidine
kinase, cannot 
activate ACV, the following mechanisms should be discussed regarding the
possible 
effectiveness of ACV in lymphoproliferative diseases: a direct cytopathic
effect; activation 
of ACV by the thymidine kinase of viruses not yet detected in cutaneous 
lymphoproliferative disorders; ACV activation by cellular thymidine kinase,
which has 
been found to be elevated in lymphoproliferative disorders. Preliminary
clinical 
observations suggest that ACV may exhibit an antiproliferative effect
intravenously in 
some patients with lymphomatoid papulosis. 

============================================================ 
58.) Eosinophilic histiocytosis: a variant form of lymphomatoid papulosis
or a disease sui 
generis? 
============================================================ 
SO - J Am Acad Dermatol 1985 Dec;13(6):952-8 
AU - McLeod WA; Winkelmann RK 
PT - JOURNAL ARTICLE 
AB - Five patients are reported on whose clinical skin disease consisted of 
polymorphous papulonodular lesions healing with a depigmented scar.
Although all cases 
had been termed lymphomatoid papulosis after clinical or histologic
examination, the 
lesions consisted principally of masses of histiocytes and eosinophils.
Individual lesions 
healed spontaneously or with minimal treatment, but the chronic course of
disease was not 
altered by any therapy used. Follow-up 3 to 17 years later indicated
persistent or 
recurrent disease, and one patient died of histiocytic malignancy.
Eosinophilic histiocytosis 
is the microscopic picture of an unusual group of patients with chronic
papulonodular 
necrotic skin disease that may deserve to be considered a disease pattern
per se. 

============================================================ 
59.) Lymphomatoid papulosis: a premalignant T cell disorder. 
============================================================ 
SO - J Am Acad Dermatol 1985 Nov;13(5 Pt 1):736-43 
AU - Espinoza CG; Erkman-Balis B; Fenske NA 
PT - JOURNAL ARTICLE 
AB - In an attempt to better define the process of lymphomatoid papulosis,
two cases 
were studied by means of light and electron microscopy,
immunohistochemistry studies, 
including the use of monoclonal antibodies, and cytogenetic technics. About
90% of the 
dermal lymphoid infiltrate, including the atypical cells, reacted with
antibodies that define 
helper-inducer T cells. Only a few cells, about 5%, reacted with antibodies
that define 
cytotoxic-suppressor T cells. Langerhans cells were increased mostly within
the 
epidermis, and in the dermis they were in close proximity to lymphoid
cells. Cytogenetic 
studies disclosed an abnormal hypertetraploid karyotype in dividing cells
from the skin 
lesion, whereas skin fibroblast and phytohemagglutinin-stimulated cells
from peripheral 
blood cultures had a diploid karyotype. The results support the concept that 
lymphomatoid papulosis is a disorder characterized by a predominance of
helper-inducer 
T cells, including the atypical cells bearing an abnormal karyotype. 

============================================================ 
60.) Clinical and histologic differentiation between lymphomatoid papulosis
and pityriasis 
lichenoides. 
============================================================ 
SO - J Am Acad Dermatol 1985 Sep;13(3):418-28 
AU - Willemze R; Scheffer E 
PT - JOURNAL ARTICLE 
AB - The relationship between lymphomatoid papulosis and pityriasis
lichenoides is a 
matter of considerable debate. Differentiation between these two conditions
is, however, 
important because patients with lymphomatoid papulosis, unlike those with
pityriasis 
lichenoides, may develop systemic lymphoma and thus require long-term
follow-up. In 
our study the clinical and histologic features of eighty-two patients with
pityriasis 
lichenoides and twenty-six patients with lymphomatoid papulosis were
reviewed and 
compared. Clinical and histologic differences were recognized, not only
allowing 
differentiation between the two conditions, but also suggesting that they are 
pathogenetically distinct diseases. Finally, evidence is presented to
suggest that the 
different views on the relationship between these diseases mainly result
from differences in 
patient selection. 

============================================================ 
61.) The clinicopathologic spectrum of lymphomatoid papulosis: study of 31
cases. 
============================================================ 
SO - J Am Acad Dermatol 1983 Jan;8(1):81-94 
AU - Sanchez NP; Pittelkow MR; Muller SA; Banks PM; Winkelmann RK 
PT - JOURNAL ARTICLE 
AB - Herein we review the Mayo Clinic experience with thirty-one cases of 
lymphomatoid papulosis seen since 1965. All patients had chronic,
recurrent, and 
self-healing erythematous papulonodular lesions, which often became
pustular, ulcerated, 
and resolved with scarring. The clinical features often corresponded to
those seen in 
Mucha-Habermann disease; however, the predominant histopathologic feature
was an 
infiltrate composed primarily of atypical lymphoid cells suggestive of
malignant lymphoma. 
In six patients, a lymphoproliferative disorder was eventually diagnosed.
There were two 
cases of mycosis fungoides (stage I), one case of nodular sclerosing
Hodgkin's disease, 
and three cases of malignant lymphoma--one diffuse mixed large and small
cell type with 
features of T-immunoblastic type, one diffuse large cell type, and one
follicular small 
cleaved cell type. The clinical course of the lymphomatoid papulosis was
unaffected by 
chemotherapy for the lymphoproliferative disorder. Our data indicate that,
with sufficient 
duration of follow-up, malignant lymphoma may develop in some patients with 
lymphomatoid papulosis. 

============================================================ 
62.) [Lymphomatoid papulosis in a child] 
============================================================ 
SO - Hautarzt 1993 Oct;44(10):674-9 
AU - Milde P; Goerz G; Lehmann P 
PT - JOURNAL ARTICLE; REVIEW (25 references); REVIEW OF REPORTED 
CASES 
AB - We report a case of a 3-year-old boy who developed crops of papules and 
ulcerating nodules on the limbs in April 1992. Periodically, new lesions
continue to erupt, 
while others resolve spontaneously. This course is characteristic for
rhythmic paradoxical 
eruptions. This course and the clinical picture, supported by the
histopathological and 
immunohistochemical findings, led to the diagnosis of lymphomatoid papulosis. 
Lymphomatoid papulosis is extremely rare in childhood. All published cases of 
lymphomatoid papulosis in children under 10 years of age are reviewed. The
differential 
diagnosis of lymphomatoid papulosis in childhood includes arthropod
assaults, pityriasis 
lichenoides et varioliformis acuta, primary cutaneous Hodgkin's disease,
Ki-1 large cell 
anaplastic lymphoma and other lymphomas and pseudolymphomas in children. 

============================================================ 
63.)Lymphomatoid papulosis in an 11-month-old infant. 
============================================================ 
SO - Pediatr Dermatol 1984 Oct;2(2):124-30 
AU - Rogers M; de Launey J; Kemp A; Bishop A 
PT - JOURNAL ARTICLE 
AB - Lymphomatoid papulosis was seen in an 11-month-old child. The condition 
resolved spontaneously after a course of only 8 weeks and the patient has
now been 
disease free for 9 months. Electron microscopy showed infiltrating
lymphocytes with 
cleaved nuclei suggestive of T cells. Monoclonal antibody studies confirmed
the T cell 
nature of the infiltrate. In this case, suppressor (OKT8) T cells were more
prominent than 
helper (OKT4) T cells, in contrast to previous reports. 

============================================================ 
64.) Topical carmustine therapy for lymphomatoid papulosis. 
============================================================ 
SO - Arch Dermatol 1985 Nov;121(11):1410-4 
AU - Zackheim HS; Epstein EH Jr; Crain WR 
PT - JOURNAL ARTICLE 
AB - Seven patients with lymphomatoid papulosis were treated with
solutions of topical 
carmustine, a nitrosourea compound. Recently used schedules have employed
10 mg of 
carmustine in dilute alcohol applied to the total skin surface daily for
four to 17 weeks 
(total dosage, 280 to 1,180 mg). All patients experienced a rapid reduction
in the number 
and size of lesions. Maintenance therapy consisted of local applications of
carmustine (2 
to 4 mg/mL of 95% ethanol) to individual new papules. This method was
effective in 
suppressing disease activity and reduced by half the average life cycle of
individual 
lesions. However, long-term lesion-free remissions were not seen. Bone marrow 
depression did not occur. 

============================================================ 
65.)Immunohistology of pityriasis lichenoides et varioliformis acuta and
pityriasis 
lichenoides chronica. Evidence for their interrelationship with
lymphomatoid papulosis. 
============================================================ 
SO - J Am Acad Dermatol 1987 Mar;16(3 Pt 1):559-70 
AU - Wood GS; Strickler JG; Abel EA; Deneau DG; Warnke RA 
PT - JOURNAL ARTICLE 
AB - Pityriasis lichenoides et varioliformis acuta and pityriasis
lichenoides chronica are 
idiopathic, papular eruptions that exhibit certain clinicopathologic
similarities to each other 
and to lymphomatoid papulosis. In order to determine if these disorders are
also similar 
immunologically, we studied the immunopathology of five biopsy specimens
from three 
cases of pityriasis lichenoides et varioliformis acuta and three biopsy
specimens from three 
cases of pityriasis lichenoides chronica. We then compared them to our prior 
immunohistologic study of nine cases of lymphomatoid papulosis. Pityriasis
lichenoides et 
varioliformis acuta and pityriasis lichenoides chronica both exhibited a
dermal and 
epidermal infiltrate of CD4+ and CD8+ T cells expressing activation
antigens. These were 
admixed with numerous macrophages. The lesional epidermis was diffusely human 
lymphocyte antigen (HLA)-DR+ and contained decreased CD1+ dendritic cells. 
Endothelial cells were also HLA-DR+. Cells bearing the phenotypes of B
cells, follicular 
dendritic cells, or natural killer/killer cells were essentially absent.
Except for the lack of 
large atypical cells, the results resembled those described previously for
lymphomatoid 
papulosis. These findings indicate that pityriasis lichenoides chronica,
pityriasis lichenoides 
et varioliformis acuta, and lymphomatoid papulosis share several
immunohistologic 
features. Together with certain clinicopathologic similarities, they are
consistent with the 
hypothesis that these three disorders are interrelated. 

===================================================== 
DATA-MEDICOS/DERMAGIC-EXPRESS No 2-(86) 12/01/2000 DR. JOSE 
LAPENTA R. 
====================================================