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

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.


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.

[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center