title: [Sporadic superficial pemphigus in the child: 2 cases].
abstract: BACKGROUND
  Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typical clinical manifestations.
  
  
  METHODS
  Case n(o) 1. A 5-year-old girl was seen for a vesiculobullous crusted dermatosis involving the trunk and the face which had developed over the last 5 months, predominantly in periorificial and fold localizations. Histology showed intragranulous acatholysis. Direct skin immunofluorescence was positive for anti-intercellular substance IgG and C3. Indirect immunofluorescence was positive for anti-intercellular substance antibodies at 1/500. The diagnosis of superficial pemphigus was retained and the child was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/kg/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Outcome was favorable allowing withdrawal of prednisone at 18 months. Case n(o) 2. A 6-year-old had developed since the age of 18 months a generalized and polycyclic pruriginous erythemato-squamous dermatosis with oozing discharge 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 was finally obtained after repeated direct immunofluorescence tests which revealed anti-intercellular substance IgG. Indirect immunofluorescence was negative. The child was given prednisone (2 mg/kg/d).
  
  
  CONCLUSIONS
  In children, pemphigus foliaceus has an exceptional frequency and 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 negative in cases with highly suggestive clinical presentations. It would be reasonable to attempt "minor" treatments as the first line approach. Systemic corticosteroids are however the treatment of choice despite the risk of classical side effects. Childhood pemphigus foliaceus is not an attenuated clinical form of adult pemphigus. Mortality is not negligible and is close to that in adults.
authors: Service de Dermatologie, Hôpital Saint-Louis, Paris.
authors: M
authors: M
authors: Rybojad
authors: G
authors: G
authors: Ducloy
authors: J L
authors: JL
authors: Reymond
authors: I
authors: I
authors: Moraillon
authors: B
authors: B
authors: Flageul
authors: M D
authors: MD
authors: Vignon-Pennamen
authors: P
authors: P
authors: Morel
authors: E
authors: E
authors: Bourrat
mesh_terms: D000328:Adult; D002648:Child; D002675:Child, Preschool; D003622:Dapsone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005148:Facial Dermatoses / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000008:administration & dosage; D012867:Skin / Q000473:pathology
chemical_list: D003622:Dapsone; D011241:Prednisone