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伴有不寻常表现且无可检测自身抗体的副肿瘤性自身免疫性多器官综合征(副肿瘤性天疱疮)

Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) with unusual manifestations and without detectable autoantibodies.

作者信息

Sanz-Bueno Jimena, Cullen Daniella, Zarco Carlos, Vanaclocha Francisco

机构信息

Department of Dermatology, 12 de Octubre University Hospital, Madrid, Spain.

出版信息

Indian J Dermatol Venereol Leprol. 2014 Jul-Aug;80(4):328-30. doi: 10.4103/0378-6323.136898.

Abstract

We describe a patient with paraneoplastic autoimmune multiorgan syndrome (PAMS) secondary to a lymphoblastic T- cell lymphoma who presented with a lichenoid dermatitis and vitiligo, later developing bronchiolitis obliterans and autoimmune hepatitis. Notably, he had no detectable autoantibodies. The development of vitiligo and autoimmune hepatic involvement probably indicate a role for cytotoxic T- cell lymphocytes in the pathogenesis of this syndrome.

摘要

我们描述了一名继发于淋巴细胞性T细胞淋巴瘤的副肿瘤性自身免疫多器官综合征(PAMS)患者,该患者最初表现为苔藓样皮炎和白癜风,随后发展为闭塞性细支气管炎和自身免疫性肝炎。值得注意的是,他未检测到自身抗体。白癜风和自身免疫性肝脏受累的发生可能表明细胞毒性T淋巴细胞在该综合征的发病机制中起作用。

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