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[Sporadic superficial pemphigus in the child: 2 cases].

M Rybojad1, G Ducloy, J L Reymond

  • 1Service de Dermatologie, Hôpital Saint-Louis, Paris.

Annales De Dermatologie Et De Venereologie
|March 30, 1999
PubMed
Summary
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Childhood pemphigus foliaceus is rare but presents with chronic skin conditions. Early diagnosis via direct immunofluorescence is crucial for effective treatment and management.

Area of Science:

  • Dermatology
  • Immunodermatology
  • Pediatric Dermatology

Context:

  • Childhood pemphigus foliaceus is an uncommon autoimmune blistering disease.
  • Pemphigus foliaceus typically presents with superficial erosions and crusting, often affecting the face and scalp.
  • Diagnosis in children can be delayed due to atypical presentations.

Purpose:

  • To report two cases of childhood pemphigus foliaceus with typical clinical manifestations.
  • To highlight the diagnostic challenges and emphasize the role of direct immunofluorescence.
  • To discuss treatment strategies and prognosis in pediatric pemphigus foliaceus.

Summary:

  • Two children, aged 5 and 6, presented with chronic, crusted, and erosive dermatoses suggestive of pemphigus foliaceus.

Related Experiment Videos

  • Histopathology and direct immunofluorescence confirmed the diagnosis by detecting intercellular IgG deposits.
  • Treatment involved systemic corticosteroids and dapsone, with favorable outcomes but highlighting potential hematological intolerance.

    • Impact:

    • Emphasizes the importance of considering pemphigus foliaceus in children with extensive, chronic dermatoses.
    • Highlights direct skin immunofluorescence as a key diagnostic tool, even with initial negative results.
    • Suggests that childhood pemphigus foliaceus is not a milder form and carries significant mortality risks similar to adults.