播散性浅表性光线性汗孔角化病继发皮肤淀粉样变性1例并文献复习  

Disseminated superficial linear porokeratosis complicated with amyloidosis cutis: a case report and literature review

作　　者：刘辉煌 陈晓红 陈税莹 杨英 黄洋 袁伟 LIU Hui-huang;CHEN Xiao-hong;CHEN Shui-ying;YANG Ying;HUANG Yang;YUAN Wei(Department of Dermatology,Affiliated Hospilul of Zunyi Medical University,Zunyi 563003,China)

机构地区：[1]遵义医科大学附属医院皮肤科,贵州遵义563003

出　　处：《临床皮肤科杂志》2020年第11期673-676,共4页Journal of Clinical Dermatology

摘　　要：报告1例播散性浅表性光线性汗孔角化病继发皮肤淀粉样变性。患者女,69岁。躯干及四肢褐色环状斑片30年,加重伴瘙痒1年。皮肤科检查:胸部、右侧肩部及四肢可见散在分布、边界清晰的直径0.3~1.5 cm环形褐色斑片,边缘呈角化性堤状隆起,中央略萎缩凹陷。双侧小腿可见圆形或类圆形直径0.1~0.3 cm褐色丘疹,表面粗糙,质硬,其间可见大小不一的环状或不规则形褐色斑片。右肩部皮损组织病理检查:角化过度,表皮性凹窝中可见角化不全柱,下方无颗粒层,真皮浅层少量淋巴细胞浸润,可见噬色素细胞,符合播散性浅表性光线性汗孔角化病。下肢皮损组织病理检查:角化过度,未见角化不全柱,表皮轻度增生,真皮乳头层增宽,其内有强折光性、轻度嗜伊红物质,其间有裂隙,可见组织细胞和噬色素细胞浸润。刚果红染色阳性,符合皮肤淀粉样变性。诊断:播散性浅表性光线性汗孔角化病继发皮肤淀粉样变性。A case of disseminated superficial linear porokeratosis with amyloidosis cutis is reported. A 69-year-old female presented with brown ring-like patches on torso and limbs for 30 years, which was aggravated with itching for 1 year. Physical examination showed well-defined round brown spots(0.3~1.5 cm in diameter) scattered in the fore chest, right shoulder and limbs,with keratinized dike-like protrusion on the margin and slightly atrophic depression in the center. Round or round-like brown,solid papules(0.1~0.3 cm in diameter, rough surface and hard texture), and ring-shaped or irregular brown patches of different sizes were seen in her bilateral calves. Histopathological examination of the right shoulder lesion showed hyperkeratosis, incomplete keratinized column in the epidermal dimple, no granular layer below, a small number of lymphocytes infiltrating in the superficial dermis, visible phagocytes, which was consistent with the features of disseminated superficial linear poro keratosis.Histopathological examination of skin lesions in lower extremities showed hyperkeratosis, no incomplete keratinized column, mild epidermal hyperplasia, widened dermal papilla, strong refraction and mild eosinophilic substance inside, in-between cracks, infiltrated histocytes and pigmentophagocytes, and positive gangrene staining, which was consistent with the features of amyloidosis cutis. The diagnosis of disseminated superficial linear porokeratosis with amyloidosis cutis was made.

关 键 词：汗孔角化病 皮肤淀粉样变性 

分 类 号：R758.7[医药卫生—皮肤病学与性病学] R758.6[医药卫生—临床医学]