疣状肢端角化症的临床及病理特点研究  

Clinical and histopathological characteristics of acrokeratosis verruciformis

作　　者：王培光[1] 林国书[1] 高敏[1] 林达[1] 李卉[1] 朱亚刚[1] 张国龙[1] 杨森[1] 张学军[1] 

机构地区：[1]安徽医科大学第一附属医院皮肤性病科,合肥230022

出　　处：《中国麻风皮肤病杂志》2005年第11期872-874,共3页China Journal of Leprosy and Skin Diseases

摘　　要：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20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。Objective： To characterize the clinical and histopathological profiles of acrokeratosis verruciformis. Methods： The clinical and pathological data of 17 patients with aerokeratosis verrueiformis 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 elinical manifestations were brown and brownish flat- toped 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 spirelike 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 spirelike change in dennis,

关 键 词：疣状肢端角化症 病理特点 临床 乳头瘤样增生 皮损表现 发病年龄 扁平疣样 毛囊角化病 病理资料 文献报道 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]