Childhood mastocytosis: results of a single center.
OBJECTIVE
We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic.


METHODS
The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed.


RESULTS
All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1.6. The median age for onset of disease was 12.1 months and the disease occured in the newborn period in 3 patients. While all patients had eruption, 10 patients had pruritis, 1 patient had a bullous formation, 1 patient had abdominal pain and 1 patient had attacks of redness throughout the body and a sense of burning in the chest. Two patients had a positive familial history. The diagnosis was confirmed with skin biopsy in all patients. The median follow up time of the patients were 5 years. The patients were treated with H1, H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs. The lesions resolved completely in 4 patients who reached to puberty and 7 patients had marked improvement in a 5.5 year-follow-up period. Ten patients had stabile lesions in a 3.6 year-follow-up period.


CONCLUSIONS
Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis. The prognosis is good; the disease limits itself and is prone to regress in the adolescent period.
Department of Pediatrics, Division of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Gülen
G
Tüysüz
Department of Pediatrics, Division of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Nihal
N
Özdemir
Department of Pediatrics, Division of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Hilmi
H
Apak
Department of Dermatology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Zekayi
Z
Kutlubay
Department of Pathology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Cuyan
C
Demirkesen
Department of Pediatrics, Division of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Tiraje
T
Celkan
Childhood mastocytosis; cutaneous mastocytosis; mastocytoma; urticaria pigmentosa