Souza Sonia Cristina de Magalhães, Kuruma Katia Akemi Miyazato, Andrade Danieli Castro Oliveira de, Azevedo Pedro Ming, Figueiredo Camille Pinto, Borba Eduardo Ferreira, Gonçalves Célio Rodrigues, Borges Cláudia Teresa Lobato
HC, USP.
Rev Bras Reumatol. 2004 Apr;44(2):175-8. doi: 10.1590/s0482-50042004000200013.
Primary Sjögren's Syndrome (pSS) is an autoimmune disease with a large spectrum of clinical manifestations extending from an organ-specific involvement to a systemic process. The skin is affected quite commonly and the estimated frequency of inflammatory vascular lesions is from 20% to 30%. Two specific, clinically recognizable forms of cutaneous vasculitis predominate, palpable purpura and chronic urticaria, but erythema multiforme, erythema perstans, erythema nodosum, erithematous macules and subcutaneous nodules have also been described. The authors report the case of a 46-year-old female patient, diagnosed as primary SS, who presented ocular and oral symptoms, poliarthritis and laboratory alterations (with a positive ANA, anti-SSA, rheumatoid factor, and hypergammaglobulinemia). Ten years after the diagnosis, she presented leg ulcers. The biopsy confirmed the presence of vasculitic process, and the ulcers improved rapidly after the treatment with endovenous cyclophosphamide. There are only two reports of chronic ulceration of the legs as cutaneous manifestation of SS. The authors stress the importance of considering ulcers in the differential diagnosis of cutaneous involvement of primary SS.
原发性干燥综合征（pSS）是一种自身免疫性疾病，临床表现范围广泛，从器官特异性受累到全身性病变。皮肤受累较为常见，炎症性血管病变的估计发生率为20%至30%。两种特定的、临床上可识别的皮肤血管炎形式较为常见，即可触及性紫癜和慢性荨麻疹，但也有关于多形红斑、持久性红斑、结节性红斑、红斑性斑疹和皮下结节的描述。作者报告了一例46岁女性患者，诊断为原发性干燥综合征，出现眼部和口腔症状、多关节炎及实验室检查异常（抗核抗体、抗SSA、类风湿因子阳性及高球蛋白血症）。诊断十年后，她出现腿部溃疡。活检证实存在血管炎病变，经静脉注射环磷酰胺治疗后溃疡迅速好转。仅有两篇关于腿部慢性溃疡作为干燥综合征皮肤表现的报告。作者强调在原发性干燥综合征皮肤受累的鉴别诊断中考虑溃疡的重要性。
