Childhood pemphigus foliaceus: 2 cases.

Introduction. Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typic al clinical manifestations. Case reports. Case n degrees l. A 5-year-old girl was seen for a vesiculobu llous crusted dermatosis involving the trunk and the face which had develop ed over the last 5 months, predominantly in periorificial and fold localiza tions. Histology showed intragranulous acatholysis. Direct skin immunofluor escence was positive for anti-intercellular substance IgG and C-3. Indirect immunofluorescence was positive for anti-intercellular substance antibodie s at 1/500. The diagnosis of superficial pemphigus was retained and the chi ld was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/k g/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Ou tcome was favorable allowing withdrawal of prednisone at 18 months. Case n degrees 2. A 6-year-old had developed since the age of 18 months a generali zed and polycyclic pruriginous erythemato-squamous dermatosis with oozing d ischarge which started and predominated on the face (periorificial zones). Trace element (copper, selenium, zinc) and vitamin(A, E and B1) assays were within the normal range. Glucagon was normal. Histological examinations of several biopsies were non-contributive. Diagnosis of pemphigus foliaceus w as finally obtained after repeated direct immunofluorescence tests which re vealed anti-intercellular substance IgG. Indirect immunofluorescence was ne gative. The child was given prednisone (2 mg/kg/d). Discussion. In children, pemphigus foliaceus has an exceptional frequency a nd diagnosis is often made quite late (mean 8 months). The diagnosis should always be entertained in children who develop chronic extensive erythemato -squamous and crusted dermatosis, even if formation is absent. Direct skin immunofluorescence confirms the diagnosis and should be repeated if negativ e in cases with highly suggestive clinical presentations. It would be reaso nable to attempt "minor" treatments as the first line approach. Systemic co rticosteroids are however the treatment of choice despite the risk of class ical side effects. Childhood pemphigus foliaceus is not an attenuated clini cal form of adulte pemphigus. Mortality is not negligible and is close to t hat in adults.