儿童皮肤肥大细胞增生症临床病理分析

目的探讨皮肤肥大细胞增生症（CM）的临床病理和免疫组化表型特征。方法对9例CM进行临床病理观察和免疫表型染色。结果9例患儿中4例，女5例；发病年龄〈6个月5例。色素性荨麻疹7例，肥大细胞瘤1例，弥漫性皮肤肥大细胞增生症1例。皮损组织病理学检查示真皮层肥大细胞聚集。免疫组化显示肥大细胞表达CD117、CD68、CD45，不表达S-100和CD1a。Giemsa染色显示9例肥大细胞胞质内均出现数量不等的紫红色异染颗粒。结论CM多见于儿童，大多数患儿在6个月内发病，结合临床表现和皮损病理活检可确诊。

Objective To investigate the clinicopathologic features of cutaneous mastocytosis （ CM ） . Methods Nine cases of CM were studied according to the clinicopathologic observation and immunophenotyping. Results There were 4 males and 5 females in the 9 cases, 5 cases occurred within 6 months of age. Of the 9 patients, 7 had urticaria pigmentosa, 1 mastocytoma, 1 diffuse mastoeytosis. Skin biopsy demonstrated increased numbers of mast cells in the dermis. Immunohistochemistry stain revealed that mast cells expressed CD117 （ 4/5 ） , CD45 （ 6/6 ） and CD68 （ 6/6 ） . All cases showed negative stain for CDla and S-100. Purple metachromatic granules in the mast cells were demonstrated by Giemsa stain in all cases. Conclusions Cutaneous mastocytosis occurs predominantly in childhood and often in the first six months of age. The diagnosis of CM is established by clinical features and skin biopsy.