疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。

Clinical and histopathological characteristics of acrokeratosis verruciformis

Objective: To characterize the clinical and histopathological profiles of acrokeratosis verruciformis. Methods: The clinical and pathological data of 17 patients with acrokeratosis verruciformis reported in Chinese literature in the past 40 years were analyzed. Results: Ten patients (59%) had the onset of the disease before the age of 20. Ten patients (59%) had positive family history inherited as autosomal dominant pattern. Seven patients (41%) had the onset of the disease at the age >25 years. The main clinical manifestations were brown and brownish flat-topped papules, similar to verruca plana in 16 patients, distributed on dorsal sides of hands, feet, forearms, and lower legs. Keratosis in palms and soles was observed in 6 cases (35%), and nail involvement in 5 cases (29%). Three patients (18%) were also complicated by Darier's disease. The histopathological pictures showed some distinctive changes including marked hyperkeratosis, granulosis, acanthosis, papillomatosis resulting in spire-like change in epidermis. Conclusion: In most cases the onset of acrokeratosis verruciformis is before 20 years, and some patients have a positive family background. The main skin lesions are hyperkeratotic papules similar to verruca plana. The main characteristic histopathological picture includes parakeratosis, dyskeratosis, vacuolation and spire-like change in dermis.