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Pemphigus foliaceus

C Galambrun¹, F Cambazard, C Clavel

¹Hôpital Nord, Saint Etienne, France.

Archives of Disease in Childhood

|November 26, 1997

Summary

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Pemphigus foliaceus, a rare childhood skin disease, showed resistance to steroids. Dapsone effectively treated the condition in an 8-year-old boy with no relapse after treatment cessation.

Area of Science:

Dermatology
Immunology
Pediatrics

Background:

Pemphigus foliaceus is an autoimmune blistering skin disease targeting keratinocyte adhesion.
Juvenile pemphigus foliaceus is rare, lacking established treatment guidelines.
Steroid treatment failure necessitates exploring alternative therapies for pediatric cases.

Observation:

An 8-year-old boy presented with pemphigus foliaceus unresponsive to topical steroids.
High-dose oral steroid tapering led to disease exacerbation.
The patient exhibited resistance to conventional corticosteroid therapy.

Findings:

Complete resolution of pemphigus foliaceus lesions was achieved within four weeks using dapsone.
Dapsone treatment was sustained for nine months with minimal adverse effects, including transient methaemoglobinemia.

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No disease recurrence was observed during the nine-month follow-up post-dapsone discontinuation.

Implications:

Dapsone represents a viable and effective treatment option for refractory juvenile pemphigus foliaceus.
This case highlights the potential of dapsone in managing pediatric autoimmune blistering diseases.
Further research into dapsone's efficacy and safety in pediatric dermatology is warranted.