色素性荨麻疹的诊断与发病机制研究进展  

Advances in Diagnosis and Pathogenesis of Urticaria Pigmentosa

作　　者：晏亮 晏洪波 YAN Liang;YAN Hongbo(Department of dermatology,General Hospital of Central Theater Command of PLA,Wuhan 430070,China)

机构地区：[1]中国人民解放军中部战区总医院皮肤科

出　　处：《皮肤科学通报》2019年第6期684-690,9,共7页Dermatology Bulletin

摘　　要：色素性荨麻疹(urticaria pigmentosa,UP)是肥大细胞增生症中最常见的皮损类型,可分为单一形态型、多形态型。成人和儿童肥大细胞增生症患者均常有UP皮损,但临床特点和预后上有所差异。在明确有肥大细胞增生症皮肤表现的患者中,需完善相关检查排除系统性肥大细胞增生症才能诊断皮肤肥大细胞增生症(cutaneous mastocytosis,CM),CM中满足UP皮损特点即可诊断为UP。偶发的c-kit基因功能获得性突变在肥大细胞增生症的发病机制中起到了重要的作用。CM的临床治疗主要以避免和减少肥大细胞释放介质、改善症状为主。Urticaria pigmentosa is the most frequent form of the skin lesions in mastocytosis,which can be subdivided into two variants,monomorphic variant and polymorphic variant.Both adult and pediatric patients with mastocytosis often present UP lesions,while they have different clinical characteristics and prognosis.It is necessary to finally diagnose with cutaneous mastocytosis among patients with mastocytosis in the skin to exclude systemic mastocytosis after related examinations,and then the diagnosis of UP can be confirmed according to the features of lesions.The presence of somatic,activating c-kitmutations plays an important role in the pathogenesis of mastocytosis.The treatment strategies of CM are mainly to avoid relevant triggers for mast cell degranulation and use drugs aimed at mast cell mediators.

关 键 词：色素性荨麻疹 皮肤型肥大细胞增生症 肥大细胞增生症皮肤表现 KIT突变 

分 类 号：R758.24[医药卫生—皮肤病学与性病学]