Cutaneous mastocytosis in children: A children analysis of 71 cases
Por:
Kiszewski A.E., Durán-McKinster C., Orozco-Covarrubias L., Gutiérrez-Castrellón P., Ruiz-Maldonado R.
Publicada:
1 ene 2004
Resumen:
Objective. To characterized the clinical features the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children. Background. Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation is probably a clonal disease with benign prognosis. Methods. The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis. Results. 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. Conclusion. 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. © 2004 European Academy of Dermatology and Venereology.
Filiaciones:
Kiszewski A.E.:
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Mexico DF, 04530, Mexico
Durán-McKinster C.:
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Mexico DF, 04530, Mexico
Orozco-Covarrubias L.:
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Mexico DF, 04530, Mexico
Gutiérrez-Castrellón P.:
Department of Clinical Research, National Institute of Paediatrics, Insurgentes Sur 3700-C, Mexico DF, 04530, Mexico
Ruiz-Maldonado R.:
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Mexico DF, 04530, Mexico
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