Srisupalak Singalavanija MD*, Wanida Limpongsanurak MD*
Affiliation : * Dermatology Unit, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok
Background : Mastocytosis is a disorder of mast cells proliferation within various organs, most commonly in
the skin. The disease more commonly appears during infancy than adult.
Objective : To characterize the clinical features, response to therapy and prognosis of cutaneous mastocytosis
in children.
Material and Method: A retrospective study of cutaneous mastocytosis was performed at Queen Sirikit
National Institute of Child Health during January 1994 to December 2007.All cases were confirmed by
histological diagnosis.
Results : There were a total of 50 patients. The male to female ratio was 1:1.2. Age at onset of lesions ranged
from birth to 7 years. Forty-seven patients (94%) developed skin lesions within the first year of life. There were
45 cases (90%) of urticaria pigmentosa, 3 cases (6%) of mastocytoma and 2 cases (4%) of diffuse cutaneous
mastocytosis. None of the patient had a family history of cutaneous mastocytosis. Most of the children were
healthy, except the one who had germ cell ovarian tumor. Skin biopsies were performed in all cases and
revealed mast cells infiltrate in the dermis. Treatment included oral antihistamine in all cases. Oral mast cell
stabilizers were given in 6 patients (12%) and topical corticosteroids in 15 patients (30%). Four patients
(8%) were treated with oral prednisolone. The skin lesions resolved only in 1 patient (2%) at age 7.8 years,
the others still had skin lesions without systemic symptoms.
Conclusion : Cutaneous mastocytosis is a benign disease in children without systemic involvement.
Keywords : Mastocytosis, Cutaneous, Thai children
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