An infant with bullous pemphigoid

Dermatol Online J. 2007 Jul 13;13(3):17.

Abstract

Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. However, it is very rare in the pediatric population. A 5-month-old male infant presented with multiple bullae located on palms and soles. Complete blood count revealed relative eosinophilia . A biopsy revealed a subepidermal blister present along with a dermal mononuclear infiltrate rich in eosinophils. In focal areas collagen flame figures, were also observed. Direct immunofluorescence showed linear reactivity for C3c and IgG at the dermal-epidermal junction. IgA was not found. Complete resolution was achieved after oral deflazacort (1.5mg/kg/day) with progressive dose reduction over 2 weeks. Bullous pemphigoid is extremely rare in the infant. In our case the disease presented with an unusual localized (acral) clinical distribution and a favorable outcome.

Publication types

  • Case Reports

MeSH terms

  • Administration, Oral
  • Administration, Topical
  • Antibodies, Anti-Idiotypic / immunology
  • Diagnosis, Differential
  • Dose-Response Relationship, Drug
  • Fluorescent Antibody Technique, Indirect
  • Follow-Up Studies
  • Glucocorticoids / administration & dosage
  • Humans
  • Immunoglobulin G / immunology
  • Immunosuppressive Agents / administration & dosage
  • Infant
  • Male
  • Pemphigoid, Bullous / diagnosis*
  • Pemphigoid, Bullous / drug therapy
  • Pregnenediones / administration & dosage
  • Recurrence
  • Skin / immunology
  • Skin / pathology

Substances

  • Antibodies, Anti-Idiotypic
  • Glucocorticoids
  • Immunoglobulin G
  • Immunosuppressive Agents
  • Pregnenediones
  • deflazacort