色素性荨麻疹(UP)是以皮肤肥大细胞增生为特征:皮诊一般持续一段时间后逐渐消退。通常在儿童期散在发生,很少在一个家族中有2名或2名以上成员发病。本文报导一家族有4人发病并综合文献中47个家族发病情况对本病进行了讨论。 本文报导的家族中第一代3人和第二代1人患有UP,发病年龄均在2～3岁,皮损分布在躯干、颈部、四肢等部位,主要为皮肤阵发性潮红及荨麻疹样皮损,伴有瘙痒,皮损随年龄增长而逐渐减轻以至消失,患者均无明显系统性表现。体检:皮损处可见扁平或微高起的 Pigmented urticaria (UP) is characterized by hyperplasia of the skin mast cells: the skin usually disappears gradually after a period of time. Occurs usually in childhood, and rarely occurs in two or more members of a family. This article reports the incidence of four families in one family and the incidence of 47 families in the comprehensive literature. This article reports the first generation of three families and one second generation with UP, the age of onset in 2 to 3 years old, lesions distributed in the trunk, neck, limbs and other parts, mainly for the skin paroxysmal flushing and Urticaria-like lesions, accompanied by itching, lesions gradually diminish with age and even disappear, patients had no obvious systemic manifestations. Physical examination: Lesions visible at the flat or slightly elevated