以臀部皮损为著的汗孔角化症一例

A case of porokeratosis with scaly plaques on the buttocks as the main manifestation

52岁男性患者,双前臂皮损3年,累及臀部、双下肢2个月。皮肤科情况:双前臂、腰部散在黄豆大褐色斑疹,边缘堤状隆起,双侧臀部密集粟粒至黄豆大褐色扁平丘疹,部分融合呈薄斑块状,表面粗糙,伴细小鳞屑,边缘隆起,境界清楚,双下肢皮损与臀部皮损类似,但数目较少且以屈侧为主,生殖器部位未见皮损。皮损组织病理学检查:网篮状角化过度,角质层可见柱状角化不全,其下方颗粒层变薄或消失,并见角化不良细胞,部分棘层肥厚,轻度海绵水肿,真皮浅层血管周围可见散在少量淋巴细胞、嗜酸粒细胞、噬黑素细胞。结合临床诊断为汗孔角化症。治疗:予口服阿维A胶囊1个月,患者因不良反应停药,皮损未见明显好转。

A 52-year-old male patient with a rash on both forearms for 3 years,involving both buttocks and lower limbs for 2 months.Physical examination showed that large brown soybean-sized rashes with levee-like ridges scattered on both forearms and waist.On both buttocks,there were brownish hyperkeratotic verrucous plaques with well-demarcated satellite papules,partially fused into thin plaques with a rough surface and small scales.The edges were raised with a clear boundary.Rashes on both lower limbs were similar to those on the buttocks,and the eruptions,with no involvement of the genital region,were predominantly located on the flexural surfaces.The histopathology of the rash showed basketweave hyperkeratosis,columnar parakeratosis of the stratum corneum,multiple coronoid lamellae,and disappearance of the granular layer underneath.There was partial acanthosis and mild spongiosis.Few lymphocytes,eosinophils,and melanophages were present around the superficial dermal vessels.A diagnosis of porokeratosis was made.The patient was recommended oral acitretin capsules but discontinued oral medication after 1 month due to adverse reactions.The follow-up is ongoing,and the lesions did not improve significantly.