小儿硬肿病1例及家系调查并文献复习

The Family Survy to a Case of Patient with Children Scleredema and the Literature Reviewed

目的 探讨小儿硬肿病及家族聚集现象。方法 报告1例小儿硬肿病的临床特点及其家族聚集现象，并通过复习文献，分析该病的临床特点、预后及治疗方法。结果 8岁患儿，因尿少、尿频1个月，皮肤发硬肿胀5天入院。经皮肤病理活检等检查后确诊为硬肿病，予改善微循环等治疗后8天症状消失，进一步家系调查发现该家系四代30人中共11人类似发病。结论 硬肿病的临床特点是对称性、非凹陷性皮肤硬肿，无痛感或触痛，最初发生在面部皮肤及颈后两侧，逐渐向肩、臂、上胸发展，最后波及腹、臀、腿，也可局限于大腿，阿辛蓝染色呈蓝色，可有家族聚集现象。

objective to explore the family aggregation in Children scleredema, methods a case of patient with children scleredema and the family were introduced and the literature about children scleredema were reviwed. Results the child aged 8 who admired with oliguria and thamuria for a month and hardening and thickening of the skin for 5 days was finally diagnosed for scleredema by skin biopsy, his symptoms were dispeared in 8 days when he was treated in by drugs of improving the microcirculation, but 11 persons were founded that have this disease through the family survy. Conclusions Family aggregation can be founded in Children scleredema.