A case of epidermolysis bullosa acquisita with clinical features of Brunsting-Perry pemphigoid showing an excellent response to colchicine - 07/08/11
, Bungo Ohyama, MD a, Shinichiro Yasumoto, MD a, Takashi Oono, MD b, Keiji Iwatsuki, MD b, Susanne Elfert c, Anja Fritsch, PhD c, Leena Bruckner-Tuderman, MD c, Takashi Hashimoto, MD a
Reprint requests: Teruki Dainichi, MD, Department of Dermatology, Kurume University School of Medicine, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan.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.
Le texte complet de cet article est disponible en PDF.Key words : Brunsting-Perry pemphigoid, epidermolysis bullosa acquisita, type VII collagen
Plan
| Supported by: a Grant-in-Aid for Scientific Research from and an Open Research Center Project of the Ministry of Education, Culture, Sports, Science, and Technology of Japan; a grant from the Ministry of Health, Labor, and Welfare (Research on Measures for Intractable Diseases 2008); the Uehara Memorial Foundation; and the Nakatomi Foundation. |
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| Conflicts of interest: None declared. |
Vol 61 - N° 4
P. 715-719 - octobre 2009 Retour au numéro
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