TY - JOUR
T1 - A case of epidermolysis bullosa acquisita with clinical features of Brunsting-Perry pemphigoid showing an excellent response to colchicine
AU - Tanaka, Noriko
AU - Dainichi, Teruki
AU - Ohyama, Bungo
AU - Yasumoto, Shinichiro
AU - Oono, Takashi
AU - Iwatsuki, Keiji
AU - Elfert, Susanne
AU - Fritsch, Anja
AU - Bruckner-Tuderman, Leena
AU - Hashimoto, Takashi
PY - 2009/10/1
Y1 - 2009/10/1
N2 - 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.
AB - 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.
KW - Brunsting-Perry pemphigoid
KW - epidermolysis bullosa acquisita
KW - type VII collagen
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U2 - 10.1016/j.jaad.2008.12.020
DO - 10.1016/j.jaad.2008.12.020
M3 - Article
C2 - 19679376
AN - SCOPUS:69949173611
VL - 61
SP - 715
EP - 719
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 4
ER -