Kiszewski A E, Durán-Mckinster C, Orozco-Covarrubias L, Gutiérrez-Castrellón P, Ruiz-Maldonado R
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Col. Insurgentes-Cuicuilco, Delegación Coyoacán, Mexico DF, CP 04530.
J Eur Acad Dermatol Venereol. 2004 May;18(3):285-90. doi: 10.1111/j.1468-3083.2004.00830.x.
To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children.
Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation, is probably a clonal disease with benign prognosis.
The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis.
There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8 : 1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease.
The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed.
描述儿童皮肤肥大细胞增多症的临床特征、对治疗的反应、病情演变及预后。
儿童肥大细胞增多症并非由潜在致癌的c-kit突变诱发，可能是一种预后良好的克隆性疾病。
分析71例肥大细胞增多症患儿的临床病理特征、病情演变及对治疗的反应。
色素性荨麻疹53例（75%），肥大细胞瘤12例（17%），弥漫性皮肤肥大细胞增多症6例（8%）。92%的病例在出生后第一年发病。男性居多，男女比例为1.8:1。治疗未改变疾病的演变过程。80%的患者病情改善或自行缓解。
肥大细胞增多症最常见的临床类型是色素性荨麻疹，其次是肥大细胞瘤和弥漫性皮肤肥大细胞增多症。94%的病例有 Darier 征。Darier 征阴性不能排除肥大细胞增多症。与成人不同，儿童肥大细胞增多症通常病程良性，无需进行复杂或侵入性的诊断检查。本文提出了儿童皮肤肥大细胞增多症的分类方法。
