three year- female had abnormalities of hair scalp ,severe pruiritis on her body and scaly erythematous skin
Erythroderma at birth
No similar family story
Netherton first described a case of (( bambo hairs )) and ichthyosis in 1958
triad : Ichthyosis- Hair shaft abnormalities - Atopy
Netherton syndrome present diagnostic challenge in infants because the trial don’t developed before the age 1 year .
At birth an exfolative erythroderma with caseous scale may be present . These children are often misdiagnosed as having other ichtyotic or erthrodermic disorder such as erythrodermic psoriasis , acrodermatitis enteropathica , linear disease , or sever atopic dermatitis
:Neonatal and infantile erythroderms retrospective study of 51 patient
Immunodeficiency 30% -
Simple or complex ichthyosis 24% -
Netherton syn 18% -
-Eczematous or papulosquamous dermatitis 20%
- patient 10 % unknown
Histology exam confirmed the diagnosis only 19 ( 45 %) of 42 cases
Hair shaft abnormalities:
Are not present at birth and don’t develop until the child is beyond infancy
Examination of eyebrow hair can improve the diagnosis of N.S better than hair scalp.
Trichorrhexis invaginata ( TI) , Trichohexsis nodosa , and pili torti
referances
November 2001 Vol. 137 No. 11 ARCHIVE OF DERMATOLOGY
Arch dermatol. 2001 ;137:747-750
Arch dermatol 2000;136:875-880