皮肤肥大细胞瘤12例分析

Clinical and pathological analysis of cutaneous mastocytoma： report of 12 cases

目的 探讨皮肤肥大细胞瘤的临床和病理特点.方法 收集12例皮肤肥大细胞瘤患儿的临床和组织病理资料.结果 12例患儿,男7例,女5例,平均年龄6.8个月,平均病程6.5个月.11例出生时即有皮疹,主要位于躯干和四肢.皮疹均为大小不一的棕褐色或橘黄色斑块、结节,单发或多发,6例病程中出现水疱,1例发生溃疡.10例皮损Darier征阳性.12例均行组织病理检查,结果显示,真皮和皮下组织密集分布许多大小、形态较均一的肥大细胞浸润,甲苯胺蓝染色阳性,免疫组化CD117阳性.3例伴较多的嗜酸性粒细胞浸润,并见表皮下均有水疱形成.其中1例可见“火焰现象”.治疗多采取口服抗组胺药等对症治疗.10例随访2～6年,皮损均变小或消退,局部遗留色素沉着或色素减退斑.结论 肥大细胞瘤的诊断主要依靠临床和组织病理检查.儿童肥大细胞瘤预后良好,数年后大多数会自动消退.

Objective To investigate the clinical and histopathological features of cutaneous mastocytoma.Methods Clinical and histopathological data were collected from 12 patients with cutaneous mastocytoma.Results Among the 12 patients,there were 7 males and 5 females with an average age of 6.8 months.The average course of disease was 6.5 months.Eleven patients presented with skin lesions at birth,which were mainly located on the trunk and extremities.Skin lesions were single or multiple,brown or orange-colored plaques and nodules of different sizes.Vesicles appeared in 6 patients,and ulcers in 1 patient during disease progression.Darier＇s sign was positive in 10 patients.Histopathological examination was carried out in 12 patients,and showed a dense infiltration of many mast cells in uniform size and shape in the dermis and subcutaneous tissue,which were stained with toluidine blue and positive for CD117.Three cases showed substantial eosinophilic infiltration with the formation of subepidermal vesicles.＂Flame figures＂ were seen in 1 patient.The patients usually received symptomatic treatment with antihistamines.Skin lesions became smaller or subsided leaving local hyperpigmentated or depigmentated macules in all the 10 patients who received 2-6 years of follow up.Conclusions The diagnosis of mastocytoma is mainly based on clinical and pathological examination.Most cases of childhood mastocytoma have a favorable prognosis,and can disappear spontaneously after several years.