毛囊型汗孔角化病8例临床病理分析

Clinicopathological analysis of eight cases of follicular porokeratosis

目的分析毛囊型汗孔角化病的临床及病理特点。方法回顾分析2015年1月至2022年2月在西京皮肤病医院确诊的8例毛囊型汗孔角化病患者的临床及病理学资料。结果8例患者发病年龄6~67岁,男6例,女2例,均无明显发病诱因,发病至确诊时间6个月至20年不等。患者皮损表现为多发或单发、直径较小(<1 cm)且长期存在的红褐色圆形斑丘疹或斑块,附少许或无鳞屑。患者多无自觉症状,偶有轻度瘙痒。4例行皮肤镜检查,均显示角栓全部发生于毛囊开口处。8例皮损组织病理表现相似:表皮厚度大致正常或棘层轻度不规则增生,仅毛囊漏斗部存在角化不全柱,其下方颗粒层变薄或消失,可见角化不良细胞。6例患者分别采用手术切除、外用药物和激光灼烧等治疗,随访结果显示,手术切除可治愈原发皮损,但无法预防新发皮损,而其他治疗方法效果不佳。结论毛囊型汗孔角化病是汗孔角化病的一种特殊病理类型,临床好发于男性,以面部多见,病情缓慢进展,组织病理表现为独特的、仅发生于毛囊口的角化不全柱,诊断需同时结合临床与病理特征。

Objective To analyze clinical and pathological features of follicular porokeratosis.Methods Clinical and histopathological data were collected from 8 patients who were diagnosed with follicular porokeratosis in Department of Dermatology,Xijing Hospital,the Fourth Military Medical University from January 2015 to February 2022,and analyzed retrospectively.Results Among the 8 patients,6 were males and 2 were females.Their average age at onset was 36 years(range,6-67 years),and the average duration from onset to diagnosis was 6 years(range,0.5-20 years).No obvious precipitating factors were found.The patients presented with multiple or solitary,small(diameter<1 cm),round,long-standing stable reddish-brown maculopapules or plaques with few or no scales on their surface.Most patients had no subjective symptoms,but for some occasional mild itching sensation.Dermoscopic examination of 4 patients showed that keratotic plugs were all present at the opening of hair follicles.Histopathological manifestations of the 8 skin lesions were similar:roughly normal epidermal thickness or mildly irregular acanthosis,parakeratotic columns only at the infundibulum of hair follicles,and thinner or disappeared granular layer containing some dyskeratotic cells.Of the 8 patients,6 were treated with surgical excision,topical drugs,or laser cautery,etc.The follow-up results showed that surgical excision could cure the primary skin lesions,but could not prevent new skin lesions,and other treatments were basically ineffective.Conclusions Follicular porokeratosis,a special type of porokeratosis,mostly occurs on the facial area of middle-aged males.This disease usually progresses slowly,and is histopathologically characterized by unique parakeratotic columns only present at the opening of hair follicles.The diagnosis of follicular porokeratosis should rely on both clinical manifestations and histopathological findings.