CAJ | 학술논문

原发性干燥综合征（primary Sjgren’s syndrome，pSS）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 pSS 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。
원발성간조종합정（primary Sjgren’s syndrome，pSS）기병은닉，림상표현다양，루진솔화오진솔교고。본병상루급인체다개기관화계통，여폐、신、혈액계통등이득도림상의생적중시，이 pSS 반발적피부점막손해보도교소。본문장원발성간조종합정반발상견피부점막손해적림상표현특점、분류、궤제급치료작이총결，이제고림상의사적인식화중시。환자피부점막손해가분위비혈관염성화혈관성손해，피손이자전양피진、뢰낙현상、구강점막궤양화불규칙피진위다，부분환자유발생피부림파류적가능。피부점막손해적궤제상불명학，여궤체자신면역내수피타파도치면역은태실형유중요관계，피부혈관염환자병변국부면역세포침윤화염성상관인자과도표체，재피부혈관염적발병중기착비상중요적작용。침대간조종합정적비엄중계통손해자，목전이대증치료위주。제고본병반발피부점막손해적인지화중시，유조우침대성치료이경호지제고환자생활질량。
The form of disease onset of primary Sjgren’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.