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

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• 作者：Noriko Tanaka ; Teruki Dainichi ; Bungo Ohyama ; Shinichiro Yasumoto ; Takashi Oono ; Keiji Iwatsuki ; Susanne Elfert ; Anja Fritsch ; Leena Bruckner-Tuderman ; Takashi Hashimoto
• 关键词：Brunsting-Perry pemphigoid ; epidermolysis bullosa acquisita ; type VII collagen
• 刊名：Journal of the American Academy of Dermatology
• 出版年：2009
• 出版时间：October 2009
• 年：2009
• 卷：61
• 期：4
• 页码：715-719
• 全文大小：569 K

文摘

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.