Format

Send to

Choose Destination
Br J Dermatol. 2015 Mar;172(3):642-51. doi: 10.1111/bjd.13567. Epub 2015 Feb 8.

Paediatric mastocytosis: a systematic review of 1747 cases.

Author information

1
Service de Dermatologie de l'Adulte et de l'Enfant, Faculté de Médecine et AP-HP, Hôpital Necker-Enfants Malades, Centre de Référence des Mastocytoses, CEREMAST, Université Paris Descartes, Paris Sorbonne Cité, 149 Rue de Sèvres, 75743, Paris Cedex 15, France.

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.

PMID:
25662299
DOI:
10.1111/bjd.13567
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center