Rongioletti F, Rebora A
Clinique Dermatologique, Université de Gènes, Italie.
Presse Med. 1996 Apr 13;25(13):631-6.
Cutaneous mucinoses are a heterogeneous group of disorders in which mucin accumulates in the skin or in the follicles. Mucin is a gelatinous substance composed of glycosaminoglycanes, especially hyaluronic acid and dermatan sulfate bound to small quantities of chondoitin sulfate and heparin sulfate. Though the causes of mucinoses remain unknown, they can be divided into distinctive cutaneous (primary) mucinoses, in which mucin deposition is the distinctive histological sign resulting in clinically distinctive lesions, and cutaneous disorders, in which mucin deposition is an epiphenomenon (secondary mucinoses). Histologically, mucin is recognized after special staining techniques using alcian blue and colloidal iron. The microscopic localization of the mucin deposit is used to distinguish between dermal and follicular forms of primary mucinoses and between epidermal, dermal and follicular forms of secondary mucinoses. We present here the clinical and histological features of primary cutaneous mucinoses and an updated classification. The main therapeutic schemas are outlined.
皮肤黏蛋白沉积症是一组异质性疾病,其中黏蛋白在皮肤或毛囊中蓄积。黏蛋白是一种由糖胺聚糖组成的凝胶状物质,尤其是与少量硫酸软骨素和硫酸乙酰肝素结合的透明质酸和硫酸皮肤素。尽管黏蛋白沉积症的病因尚不清楚,但可分为独特的皮肤(原发性)黏蛋白沉积症,其中黏蛋白沉积是独特的组织学特征,导致临床上独特的病变,以及皮肤疾病,其中黏蛋白沉积是一种附带现象(继发性黏蛋白沉积症)。在组织学上,使用阿尔辛蓝和胶体铁的特殊染色技术后可识别黏蛋白。黏蛋白沉积物的微观定位用于区分原发性黏蛋白沉积症的真皮和毛囊形式,以及继发性黏蛋白沉积症的表皮、真皮和毛囊形式。我们在此介绍原发性皮肤黏蛋白沉积症的临床和组织学特征以及最新分类。概述了主要的治疗方案。