弥漫性色素沉着伴点状色素减退10例临床分析

Diffuse hyperpigmentation with guttate depigmentation macules： clinical analysis of 10 cases

目的 探讨弥漫性色素沉着伴点状色素减退的临床特点及诊断要点.方法 回顾分析2005-2012年收治的10例弥漫性色素沉着伴点状色素减退患者的临床表现,病理特征及转归.结果 10例患者中,男6例,女4例;其中成年患者3例,中位发病年龄7岁（4 ～ 25岁）,所有患者均无家族史.典型临床表现为弥漫、均匀的色素沉着基础上发生密集点状白斑.皮损可累及全身,无自行消退趋势.组织学均表现为表皮基底层色素略深,真皮浅层血管周围散在色素颗粒和噬色素细胞.部分基底层可见灶性空泡变性.结论 弥漫性色素沉着伴点状色素减退是一种少见的色素沉着和色素减退伴发的色素异常性疾病,常见于儿童,病理表现为炎症后色素沉着.皮损一旦发生,无消退趋势,目前尚无有效治疗方法.

Objective To assess the clinical feature and diagnosis of diffuse hyperpigmentation with guttate depigmentation macules.Methods A retrospective study was carried out among 10 patients with diffuse hyperpigmentation with guttate depigmentation macules collected at the Institute of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College from 2005 to 2012.The clinical manifestations,pathological findings and disease outcomes in these patients were analyzed.Results Of the 10 patients,6 were male,and 4 were female.The median age at onset was 7 years （range,4-25）,and there were only 3 adult patients among these patients.None of the patients had a family history of pigmentary disturbance.Typical clinical manifestations included densely distributed,guttate hypopigmented macules arising on diffuse and uniform hyperpigmentation.Lesions could slowly spread over the body surface without the trend towards spontaneous regression.Pathologically,there was a slight increase in pigmentation of the epidermal basal layer,as well as melanins and melanophages scattered around blood vessels in the superficial dermis,with or without focal vacuolar degeneration of the basal cell layer.Conclusions Diffuse hyperpigmentation with guttate depigmentation macules,a rare pigmentary disturbance that clinically manifests as both hyperpigmentation and hypopigmentation and is pathologically characterized by postinflammatory hyperpigmentation,often affects children.Once the lesions occur,there is no trend towards regression.No effective treatment is available for this entity at present.