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Juvenile pemphigus foliaceus

M Mehravaran1, M Morvay, K Molnár

  • 1Department of Dermatology, Albert Szent-Györgyi Medical University, H-6701 Szeged, Korányi fasor 6, PO Box 480, Szeged, Hungary. ME@derma.szote.u-szeged.hu

The British Journal of Dermatology
|October 10, 1998
PubMed
Summary
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Pemphigus foliaceus in a child presenting with skin lesions was diagnosed using immunofluorescence and serological tests. Successful treatment with prednisolone and dapsone led to a 3-year lesion-free remission.

Area of Science:

  • Dermatology
  • Immunology

Background:

  • Pemphigus foliaceus is a rare autoimmune blistering disease.
  • Childhood pemphigus foliaceus is exceptionally uncommon.

Observation:

  • A 7-year-old girl presented with generalized erythematous, scaling plaques and vesiculobullous lesions.
  • Direct immunofluorescence showed intercellular IgG, IgA, and C3 deposition in lesional skin.
  • Enzyme-linked immunosorbent assays detected antibodies against desmoglein 3 and desmoglein 1 epitopes.

Findings:

  • Despite negative indirect immunofluorescence for circulating antibodies, the patient was diagnosed with pemphigus foliaceus.
  • Treatment with systemic prednisolone and dapsone resulted in complete remission.
  • The patient remained lesion-free for 3 years on low-dose dapsone therapy.

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Implications:

  • This case highlights the importance of considering pemphigus foliaceus in pediatric patients with unusual skin manifestations.
  • It underscores the diagnostic utility of direct immunofluorescence and targeted serological assays.
  • The successful management demonstrates the efficacy of combined immunosuppressive and anti-inflammatory therapy in childhood pemphigus foliaceus.