季节性对称性色素性苔藓样肢端角化症215例临床分析

Clinical Analysis of 215 Patients with Seasonal Symmetric Pigmented and Lichenoid Acrokeratoderma

目的对215例季节性对称性色素性苔藓样肢端角化症患者进行临床分析,归纳总结其临床特点,探讨其防治措施。方法追踪随访、分析总结本科门诊2006年3月-2021年1月期间215例患者临床资料,比较不同治疗干预的防治效果。结果215例患者中男189例,女26例;202例无家族史,13例有家族史(5个家系);来本科首次就诊时平均年龄(24.8±11.9)岁,平均发病年龄(21.3±8.5)岁。本病春末夏初发病或复发,夏季加重,秋末冬初消失或显著减轻;皮损对称性分布于手腕、手背、踝部、膝部、足背、肘部、手侧缘、指蹼、头部,表现为色素性苔藓样角化性斑片或斑块;无明显不适。真菌学检查(-)。水桶征试验(+)。组织病理主要表现为表皮角化过度、棘层肥厚、真皮浅层血管周围慢性炎症细胞浸润。口服、外用药物治疗无效,长期保持环境干燥凉爽可以预防本病复发。结论季节性对称性色素性苔藓样肢端角化症215例患者的临床表现比较独特,认为此病是一种新出现的皮肤病,目前尚无有效治疗方法,保持环境干燥凉爽是较有效的预防方法。

Objective To analyze the clinical data of 215 patients with seasonal symmetric pigmented and lichenoid acrokeratoderma(SSPLA).Methods Two hundreds and fifteen SSPLA outpatients in our hospital from March 2006 to January 2021 were followed up and their clinical data were analyzed,and the outcomes of different treatmen were compared.Results Among them,there were 189 male and 26 female patients,202 cases without,and 13 cases with family history(from 5 families).The mean age at first visit in our department and at onset were(24.8±11.9)and(21.3±8.5)years old respectively.The lesions occurred or relapsed in late spring or early summer,aggravated in summer,and vanished or alleviated in late autumn or early winter.The lesions symmetrically distributed on the wrists,dorsum of hands,ankles,knees,dorsum of feet,elbows(cubital fossa),lateral margin of hands,fingerwebs and head.All lesions were pigmented,lichenoid,hyperkeratotic patches or plaques and without noticeable discomfort.Fungi tests were negative,and bucket sign tests in tape water were positive.The main histopathologic features were hyperkeratosis,acanthosis of the epidermis,and superficial perivascular lymphohistiocyte infiltrating in the dermis.Neither systemic treatment nor topical treatment was effective.Maintaining the environment dry and cool can prevent its recurrence.Conclusion The characteristics of SSPLA are distinct.It's a new dermatosis,and there is no effective treatment strategy,maintaining the environment dry and cool is a relatively effective prevention method.