Childhood Vulvar Mucous Membrane Pemphigoid

J Pediatr Adolesc Gynecol. 2022 Apr;35(2):171-173. doi: 10.1016/j.jpag.2021.10.010. Epub 2021 Nov 2.

Abstract

Background: Autoimmune bullous diseases in childhood are a diagnostic challenge.

Case: We present the case of an 11-year-old girl with recurrent vulvar erosions since early childhood. She had been referred to a child abuse unit under the suspicion of sexual abuse. She responded well to dapsone and topical corticosteroids.

Summary and conclusion: Our review focuses on previously reported cases of pemphigoid (bullous or mucous membrane) in childhood with exclusively genital involvement. We also summarize mucous membrane pemphigoid cases diagnosed during childhood. There seems to be a differentiated form of pemphigoid predominantly affecting girls with exclusively vulvar involvement and with good prognosis. Dermatologic evaluation and a skin biopsy with direct immunofluorescence are key to diagnosing a mucous membrane pemphigoid. Further antigenic studies are needed to nosologically classify the disease properly.

Keywords: Childhood; Genital pemphigoid; Mucous membrane pemphigoid; Vulvar pemphigoid.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Glucocorticoids
  • Humans
  • Mucous Membrane
  • Pemphigoid, Benign Mucous Membrane* / diagnosis
  • Pemphigoid, Benign Mucous Membrane* / drug therapy
  • Pemphigoid, Benign Mucous Membrane* / pathology
  • Pemphigoid, Bullous* / diagnosis
  • Pemphigoid, Bullous* / pathology
  • Vulva / pathology

Substances

  • Glucocorticoids