Rowell综合征临床观察及随访分析

Rowell＇s syndrome： a clinical observation and a follow-up analysis

目的 探讨Rowell综合征的发病特点、治疗及预后。方法 对2009年7月至2015年7月收治的6例Rowell综合征患者临床资料进行分析。结果 6例Rowell综合征患者均为女性，日晒后出现皮损2例，有用药史2例，金黄色葡萄球菌感染诱发1例，无明确诱因1例。由面部首发皮损蔓延至全身4例，双手背首发皮损蔓延至全身1例，局限于面部及双手足部1例。6例患者均出现指趾端冻疮样皮损。5例伴发热。6例患者ANA均为核颗粒型阳性，抗Sm抗体阳性4例，抗U1RNP抗体阳性5例，抗SSA抗体阳性4例，抗SSA和抗SSB抗体同时阳性1例，抗dsDNA阳性2例。组织病理表现均有角化过度，表皮轻度增生，表皮部分坏死，可见较多角化不良细胞，基底细胞液化变性；真皮浅层血管周围可见淋巴细胞浸润。入院后确诊亚急性皮肤型红斑狼疮1例，系统性红斑狼疮5例。6例患者使用泼尼松剂量为0.6 ~ 1.0 mg·kg^-1·d^-1，1例患者用过丙种球蛋白200 mg/d治疗，糖皮质激素减量过程中均加用羟氯喹0.2 g每天2次。随访1年，5例患者未再出现多形红斑或冻疮样皮损，1例失访。结论 Rowell综合征以女性多发，糖皮质激素为主要治疗药物，减量应较红斑狼疮缓慢。

Objective To investigate the characteristics, treatment and prognosis of Rowell′s syndrome. Methods Clinical data were collected from 6 inpatients with Rowell′s syndrome from July 2009 to July 2015, and retrospectively analyzed. Results All the patients were female, of them, 2 developed skin lesions after sun exposure, 2 had medication history, 1 had Staphylococcus aureus infection before occurrence of skin lesions, and 1 had no obvious precipitating factor. Skin lesions spread from the face to the whole body in 4 cases, from the dorsal hands to the whole body in 1 case, and were confined to the face, both hands and feet in 1 case. All the 6 patients had chilblain-like lesions in the digit tips, and 5 patients had fever. Among these patients, 6 were positive for antinuclear antibodies （granular pattern）, 4 for anti-Sm antibodies, 5 for anti-U1RNP antibodies, 4 for anti-SSA antibodies, 2 for anti-dsDNA antibodies, and 1 was positive for both anti-SSA and anti-SSB antibodies. Histopathological examination revealed hyperkeratinization, mild hyperplasia, partial necrosis, many dyskeratocytes, liquifaction degeneration of basal cells in the epidermis, and perivascular infiltration of lymphocytes in the superficial dermis. After admission, 1 patient was finally diagnosed with subacute cutaneous lupus erythematosus （SCLE）, and 5 with systemic lupus erythematosus （SLE）. All the 6 patients were treated with prednisone at a dose of 0.6 - 1.0 mg·kg^-1·d^-1, and 1 was also treated with gamma globulin （200 mg/d）. During the process of glucocorticoid tapering, hydroxychloroquine was administered at 0.2 g twice a day. During 1-year follow-up, neither erythema multiform nor chilblain-like lesions recurred in 5 patients, and 1 patient was lost to follow-up. Conclusions Rowell′s syndrome frequently occurs in women. Glucocorticoids are a mainstay of its treatment, and their tapering should be slower in patients with Rowell′s syndrome than in those with lupus erythematosus.