Cutaneous mastocytosis in children: a clinical analysis of 71 cases.
OBJECTIVE
To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children.


BACKGROUND
Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation, is probably a clonal disease with benign prognosis.


METHODS
The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis.


RESULTS
There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8 : 1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease.


CONCLUSIONS
The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed.
Kiszewski
A E
AE
Department of Dermatology, National Institute of Paediatrics, Insurgentes Sur 3700-C, Col. Insurgentes-Cuicuilco, Delegación Coyoacán, Mexico DF, CP 04530.
Durán-Mckinster
C
C
Orozco-Covarrubias
L
L
Gutiérrez-Castrellón
P
P
Ruiz-Maldonado
R
R
D000293:Adolescent; D017677:Age Distribution; D000704:Analysis of Variance; D001707:Biopsy, Needle; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000473:pathology*; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D008800:Mexico / Q000453:epidemiology; D011336:Probability; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D014582:Urticaria Pigmentosa / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy