A case of epidermolysis bullosa acquisita with clinical features of Brunsting-Perry pemphigoid showing an excellent response to colchicine
A case of epidermolysis bullosa acquisita with clinical features of Brunsting-Perry pemphigoid showing an excellent response to colchicine
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DOI：
10.1016/j.jaad.2008.12.020
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发表时间：
2009-10-01
影响因子：
13.8
通讯作者：
Hashimoto, Takashi
中科院分区：
文献类型：
--
作者：
Tanaka, Noriko;Dainichi, Teruki;Hashimoto, Takashi
背景：Brunsting-Perry类天疱疮是一种罕见的表皮下水疱性疾病，其特征是头部和颈部的瘢痕性水疱。然而，负责的自身抗原的身份仍然是unresolved.Methods：我们报道了一名患者与获得性大疱性表皮病谁具有典型的Brunsting-Perry类天疱疮的临床特征，并调查了所涉及的VII型胶原蛋白表位。该患者是一个65岁的日本妇女与20个月的历史，她的头，脸，和neck.Results复发表皮下大疱：免疫印迹研究表明，这名患者的血清反应与VII型胶原蛋白，特别是与非胶原结构域1和三螺旋结构域。患者完全回应秋水仙碱monetary.Limitations：这项研究进行了只有一个case.Conclusion：这项研究表明，Brunsting佩里类天疱疮可能是一个临床变异的大疱性表皮获得性。（《美国皮肤病学学会杂志》2009;61：715-9。）
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. (J Am Acad Dermatol 2009;61:715-9.)