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- Title
Paediatric mastocytosis: a systematic review of 1747 cases.
- Authors
Méni, C.; Bruneau, J.; Georgin ‐ Lavialle, S.; Le Saché de Peufeilhoux, L.; Damaj, G.; Hadj ‐ Rabia, S.; Fraitag, S.; Dubreuil, P.; Hermine, O.; Bodemer, C.
- 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 D816 V 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.
- Subjects
MAST cell disease; PEDIATRIC research; URTICARIA pigmentosa; SKIN diseases; PIGMENTATION disorders
- Publication
British Journal of Dermatology, 2015, Vol 172, Issue 3, p642
- ISSN
0007-0963
- Publication type
Article
- DOI
10.1111/bjd.13567