Classification and management of mastocytosis in childhood.

Objective. Mastocytosis is a frequently observed condition in children. We analyzed the initial manifestations and clinical course. Patients and methods. We restrospectively studied 49 cases of mastocytosis in children (29 boys and 20 girls) managed in our unit between 1985 and 199 5. All of the children had typical manifestations. Photographic documents w ere available in all cases. Results. There was pigmentary urticaria in 32 cases and a mastocytoma in 17 . Axanthelasmoid aspect and bullae were observed in some cases in both of t hese clinical forms. Complementary explorations demonstrated one case of du odenal mast cell infiltration. Excepting the case with skin and duodenal ma nifestation, all of our patients improved and clinical cure was obtained du ring growth. Discussion. The association of dermal atopia and mastocytosis does not infl uence the clinical course of these two conditions. The development of bulla e does not appear to be a Factor of poor prognosis. The xanthelasmoid aspec t of the lesions and the similar course in childhood mastocytosis and juven ile xanthogranulma would suggest that a common process with a histological spectrum including mastocytoma and xanthoma is involved. In our experience, counselling against the use of anti-cough medicines containing codeine is an essential part of management. Antihistamine agents may be prescribed for pruritis.