A case of epidermolysis bullosa acquisita with clinical features of Brunsting-Perry pemphigoid showing an excellent response to colchicine

J Am Acad Dermatol. 2009 Oct;61(4):715-9. doi: 10.1016/j.jaad.2008.12.020. Epub 2009 Aug 13.

Abstract

Background: Brunsting-Perry pemphigoid is a rare subepidermal blistering disease characterized by scarring blisters on the head and neck. However, the identity of the responsible autoantigens is still unresolved.

Methods: We reported a patient with epidermolysis bullosa acquisita who had clinical features typical of Brunsting-Perry pemphigoid and investigated the involved type VII collagen epitopes. The patient was a 65-year-old Japanese woman with a 20-month history of recurrent subepidermal bullae on her head, face, and neck.

Results: Immunoblot studies revealed that the serum of this patient reacted with type VII collagen, specifically with the noncollagenous domain 1 and the triple-helical domain. The patient responded completely to colchicine monotherapy.

Limitations: This study was performed on only one case.

Conclusion: This study suggests that Brunsting-Perry pemphigoid may be a clinical variant of epidermolysis bullosa acquisita.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Colchicine / administration & dosage*
  • Collagen Type VII / immunology
  • Epidermolysis Bullosa Acquisita / drug therapy*
  • Epidermolysis Bullosa Acquisita / immunology
  • Epidermolysis Bullosa Acquisita / pathology*
  • Epitopes / immunology
  • Female
  • Humans
  • Pemphigoid, Bullous / drug therapy*
  • Pemphigoid, Bullous / immunology
  • Pemphigoid, Bullous / pathology*
  • Tubulin Modulators / administration & dosage*

Substances

  • Collagen Type VII
  • Epitopes
  • Tubulin Modulators
  • Colchicine