Cutaneous mastocytosis in Thai children.
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.


METHODS
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 ife. 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.


CONCLUSIONS
Cutaneous mastocytosis is a benign disease in children without systemic involvement.
Singalavanija
Srisupalak
S
Dermatology Unit, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok, Thailand. srisupalak@yahoo.com
Limpongsanurak
Wanida
W
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006633:Histamine Antagonists / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000473:pathology*; D054705:Mastocytoma, Skin / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D013785:Thailand / Q000453:epidemiology; D014582:Urticaria Pigmentosa / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology
D000893:Anti-Inflammatory Agents; D006633:Histamine Antagonists; D011239:Prednisolone