原发性干燥综合征皮肤黏膜损害

Damage of Skin and Mucosamembrane in Primary Sjgren's Syndrome

原发性干燥综合征(primary Sjgren’s syndrome,p SS)起病隐匿,临床表现多样,漏诊率和误诊率较高。本病常累及人体多个器官和系统,如肺、肾、血液系统等已得到临床医生的重视,而p SS伴发的皮肤黏膜损害报道较少。本文将原发性干燥综合征伴发常见皮肤黏膜损害的临床表现特点、分类、机制及治疗作以总结,以提高临床医师的认识和重视。患者皮肤黏膜损害可分为非血管炎性和血管性损害,皮损以紫癜样皮疹、雷诺现象、口腔黏膜溃疡和不规则皮疹为多,部分患者有发生皮肤淋巴瘤的可能。皮肤粘膜损害的机制尚不明确,与机体自身免疫耐受被打破导致免疫稳态失衡有重要关系,皮肤血管炎患者病变局部免疫细胞浸润和炎性相关因子过度表达,在皮肤血管炎的发病中起着非常重要的作用。针对干燥综合征的非严重系统损害者,目前以对症治疗为主。提高本病伴发皮肤黏膜损害的认知和重视,有助于针对性治疗以更好地提高患者生活质量。

The form of disease onset of primary Sjogren＇s syndrome （pSS） is insidious, and its clinical manifestations are diverse. The rate of misdiagnosis is high. PSS often involves multiple organs and systems, such as lung, kidney, hematological system, however, reports on the damage of skin and mucosa- membrane in PSS are rare. This article reviews the common clinical manifestations of skin and mucous membrane damage, its classification, mechanism and treatment in PSS. Damage of skin and mucous membrane can be divided into vascular inflammation and vascular damage. Skin and mucous membrane damage can be purpura rash, Raynaud＇ s phenomenon, oral mucosal ulcers and irregular skin rashes. The mechanism of skin and mucous membrane damage is not clear, skin vasculitis with partial immune cell infiltration, and inflammation play very important role. To improve patients＇ quality of life, dermatologists and the internist should pay more attentions to the damage of skin and mucosa membrane in PSS.