Paediatric mastocytosis: a systematic review of 1747 cases
British Journal of Dermatology2015Vol. 172(3), pp. 642–651
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C. Méni, Julie Bruneau, Sophie Georgin‐Lavialle, L. Le Sachè de Peufeilhoux, Gandhi Damaj, S. Hadj‐Rabia, Sylvie Fraïtag, Patrice Dubreuil, Olivier Hermine, Christine Bodemer
Abstract
Paediatric mastocytosis was previously considered to be a benign and spontaneously regressing disease. However, this evolution is impossible to predict. To clarify the characteristics and course of paediatric mastocytosis, we performed a literature review of 1747 cases published between 1950 and April 2014. Lesions occurred before the age of 2 years in 90% of cases, and presented as urticaria pigmentosa (75% of cases), mastocytoma (20%) or diffuse cutaneous mastocytosis (5%). The male-to-female ratio was 1·4. KIT D816V mutation was detected in 34% of 215 tested patients. Clinical regression (complete or partial) occurred in 67% of cases and stabilization in 27%. However, the outcome was fatal in 2·9% of patients.
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