肛周生殖器部位丘疹性棘层松解性角化不良6例临床分析

Clinical analysis of six cases of papular acantholytic dyskeratosis of the anogenital area

目的分析肛周生殖器部位丘疹性棘层松解性角化不良的临床及病理特点。方法回顾性分析2013年1月至2022年12月在北京大学第三医院确诊的6例肛周生殖器部位丘疹性棘层松解性角化不良患者的临床及组织病理学资料。结果6例患者中,女3例,男3例,发病年龄22~58岁,发病至确诊时间1~18个月,均无类似皮损家族史。6例患者临床表现均为肛周或生殖器部位密集分布多发肤色或灰白色、粟粒大小丘疹,部分表面糜烂、结痂,可伴瘙痒。组织病理表现类似:角化过度,灶性角化不全,表皮内出现棘层松解及角化不良细胞。6例患者外用糖皮质激素或他克莫司软膏等治疗,疗效不一,部分皮损可持续存在,反复发作。结论丘疹性棘层松解性角化不良罕见,好发于肛周生殖器部位,诊断时需注意结合临床特征及组织病理。

Objective To analyze clinical and pathological features of 6 cases of papular acantholytic dyskeratosis of the anogenital area.Methods Clinical and histopathological data were collected from 6 patients with papular acantholytic dyskeratosis of the anogenital area in the Department of Dermatology,Peking University Third Hospital from January 2013 to December 2022,and retrospectively analyzed.Results Among the 6 patients,3 were males and 3 were females.Their age at onset ranged from 22 to 58 years.The time from the onset to diagnosis ranged from 1 to 18 months.All patients had no family history of similar skin lesions.The 6 patients all clinically presented with multiple clustered millet-sized flesh-colored or gray-whitish papules in the perianal and genital areas,with erosions and crusts on the surface of some lesions,sometimes accompanied by pruritus.Histopathological findings of skin lesions were similar,including hyperkeratosis with focal parakeratosis,as well as acantholytic and dyskeratotic cells scattered in the epidermis.The 6 patients were treated with topical glucocorticoids or tacrolimus ointment,etc.,which exhibited varying efficacy,and some skin lesions persisted with repeated attacks.Conclusions Papular acantholytic dyskeratosis was a rare skin disease,and mostly occurred in the anogenital area.Its diagnosis should rely on both clinical manifestations and histopathological findings.