Pediatric cutaneous mastocytosis: a review of 180 patients.
BACKGROUND
Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and, sometimes, other organs. Some patients may experience symptoms related to mast cell mediator release.


OBJECTIVE
To analyze the clinical features of cutaneous mastocytosis in a large series of children.


METHODS
We conducted a file review of all children clinically diagnosed with cutaneous mastocytosis in our department over the last 20 years. We evaluated gender, age at onset, character and distribution of the lesions, associated symptoms, and course of the disease.


RESULTS
Altogether, 180 patients with cutaneous mastocytosis were identified. The male to female ratio was 1.5:1. About one-third of patients had a mastocytoma, which was present at birth in over 40% and appeared during the first year of life in most of the remainder. Urticaria pigmentosa was noted in 65% of the patients, presenting at birth in 20% and during the first year in most of the remainder. The majority of lesions was distributed over the trunk and limbs. Different kinds of associated symptoms were noted. Prognosis in general was good. Only 11% of the cases, all urticaria pigmentosa, were familial.


CONCLUSIONS
Most cases of pediatric mastocytosis are sporadic and appear during the first 2 years of life, especially on the trunk. Urticaria pigmentosa is the most frequent variant. The prognosis of pediatric mastocytosis, in general, is good.
Ben-Amitai
Dan
D
Schneider Children's Medical Center of Israel, Petah Tiqva, Israel. danb@clalit.org.il
Metzker
Aryeh
A
Cohen
Herman A
HA
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007557:Israel / Q000453:epidemiology; D008297:Male; D034801:Mastocytoma / Q000453:epidemiology; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis / Q000453:epidemiology*; D011379:Prognosis; D014582:Urticaria Pigmentosa / Q000453:epidemiology