West Penn Allegheny Health System, Department of Medicine , Pittsburg, PA, USA.
J Cutan Med Surg. 2011 Jan-Feb;15(1):8-14. doi: 10.2310/7750.2010.09033.
Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.OBJECTIVE AND CONCLUSIONS:In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality.
干燥综合征（SS）是一种慢性自身免疫性炎症性疾病，主要累及外分泌腺体，导致其功能障碍。SS 通常表现为干眼（xerophthalmia）和口干（xerostomia）。这一过程可能表现为原发性 SS 的独立现象，也可能表现为另一种自身免疫过程（最常见的是类风湿关节炎或系统性红斑狼疮）背景下的继发性 SS。将近一半的 SS 患者会出现皮肤表现，包括皮肤干燥（xeroderma）、可触及和不可触及的紫癜以及/或荨麻疹样病变。这些皮肤表现被低估了，因为它们常常被更明显的干燥症状所掩盖。然而，某些皮肤表现具有至关重要的临床和预后意义，因为它们会增加发生危及生命的疾病的风险，包括多系统血管炎和非霍奇金淋巴瘤。
本文讨论了原发性 SS 的皮肤表现，重点讨论了那些预示死亡率增加的皮肤表现。
