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Case Reports
. 2009 Oct;61(4):715-9.
doi: 10.1016/j.jaad.2008.12.020. Epub 2009 Aug 13.

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

Noriko Tanaka  1 , Teruki Dainichi, Bungo Ohyama, Shinichiro Yasumoto, Takashi Oono, Keiji Iwatsuki, Susanne Elfert, Anja Fritsch, Leena Bruckner-Tuderman, Takashi Hashimoto
Affiliations
Case Reports

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

Noriko Tanaka et al. J Am Acad Dermatol. 2009 Oct.

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.

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