Department of Dermatology Zot 2400 C340 Medical Sciences 1 Irvine, CA 92697-2400, USA.
Int J Dermatol. 2011 Aug;50(8):905-14. doi: 10.1111/j.1365-4632.2011.04868.x.
The purpose of this review is to provide insight and clarification in the quandary of classification and delineate clinical and histological features and pathophysiology of paraneoplastic pemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990. Paraneoplastic pemphigus represents only one manifestation of the heterogeneous autoimmune syndrome in which patients, in addition to small airways occlusion, may display a spectrum of at least five clinical variants of the mucocutaneous disease [i.e. pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like, termed paraneoplastic autoimmune multiorgan syndrome (PAMS)]. There is a need for the expanded, inclusive classification of diverse mucocutaneous and respiratory presentations of PAMS. Multiple specific effectors of humoral and cellular autoimmunity mediating epithelial damage have been identified. An update of advances in clinical and basic research on PAMS and in management and overall prognosis of PAMS is provided.
本文旨在深入探讨副肿瘤天疱疮的分类困境，并阐述其临床和组织学特征及病理生理学。副肿瘤天疱疮是一种上皮自身免疫性和黏附性疾病，最初由 Anhalt 博士于 1990 年描述。副肿瘤天疱疮只是患者多种自身免疫综合征的一种表现形式，除了小气道阻塞外，患者还可能表现出至少五种黏膜皮肤疾病的临床变异型[即类天疱疮、类大疱性类天疱疮、多形红斑样、移植物抗宿主病样和扁平苔藓样，称为副肿瘤性自身免疫多器官综合征（PAMS）]。因此，需要对 PAMS 多样化的黏膜皮肤和呼吸表现进行扩展和包容性分类。目前已经鉴定出多种参与上皮损伤的体液和细胞自身免疫的特定效应因子。本文就 PAMS 的临床和基础研究进展、管理和总体预后方面进行了更新。
