表现为色素性对称性肢端角化性皮损的一组患者临床分析

Clinical analysis on a group of cases characterized b y pigmented symmetrical acrokeratoderma

目的报道一组表现为色素性对称性肢端角化性皮损患者的临床表现、组织病理学及治疗结果。方法系统地收集了自1998年5月至2007年9月来我科就诊的以色素性对称性肢端角化性皮损为表现的16例患者的临床资料，包括临床表现、皮损组织病理学改变、治疗、性别、年龄、职业、家族成员情况等。结果男14例，女2例；年龄9～54岁；病程2个月至8年，平均（2．0±1．5）年。主要临床表现为出现棕褐或棕黑色斑，均呈对称性斑丘疹，表面粗糙，有少许脱屑，伴轻度瘙痒。皮损部位：16例（100％）患者在双手掌指背、手腕，2例（12．5％）双肘及双膝，3例（18．8％）双踝部。组织病理学表现：表皮角化过度，棘层增厚，乳头瘤样增生；真皮少许淋巴细胞浸润。家庭成员无类似病史。给予口服维A酸类、烟酰胺、维生素B6等，外用维A酸软膏、糖皮质激素类软膏等，疗效欠佳，或病情反复。结论这是一组主要以肢端出现对称性色素性角化斑或斑丘疹，组织病理学表现以表皮角化过度、棘层增厚、乳头瘤样增生为特征的皮肤病，病因不明。

Objective To report a group of cases characterized by pigmented symmetrical acrokeratoderma. Methods Sixteen patients characterized by pigmented symmetrical acrokeratoderma were collect- ed from outpatients from May 1998 to September 2007 in the Department of Dermatology, Second Affiliated Hospital, Sun Yat-Sen University. General information on the sex, age, profession, family history of patients were documented. All patients underwent clinical and histopathological examinations, and were followed up for the assessment of treatment efficiency. Results Among these patients, 14 were males and 2 were females, ranging in age from 9 to 54 years, with duration of disease varying from 2 months to 8 years （mean course 2.0± 1.5 years ）. Characteristic manifestations were rough, scaling, brownish aubum or black maculopapular eruptions with mild pruritus. The lesions were symmetrically distributed on palms, dorsal digits and wrists of 16 （ 100% ） patients, elbows and knees of 2 （ 12.5% ） patients, and ankles of 3 （ 18.8% ） patients. Histopatho- logic examination revealed epidermal hyperkeratosis, acanthosis and papillomatous hyperplasia as well as dermal infiltration with a few lymphocytes. No heredity tendency was observed in any of the patients. The patients showed a poor response to, or experienced a relapse after treatment with oral tretinoins, nicoti- namide, vitamin B6 as well as topical tretinoin ointment, glucocorticoid ointment, and so on. Conclusion These cases may represent a new entity clinically characterized by pigmented symmetrical acrokeratoderma, and pathologically characterized by epidermal hyperkeratosis, acanthosis and papillomatous hyperplasia with unknown etiology.