Jornal de Pediatria
Print version ISSN 0021-7557
On-line version ISSN 1678-4782
FERNANDES, Evódie I. et al. Systemic mastocytosis in childhood: report of 3 cases. J. Pediatr. (Rio J.) [online]. 2002, vol.78, n.2, pp.176-180. ISSN 0021-7557. http://dx.doi.org/10.1590/S0021-75572002000200018.
Objective: mastocytosis comprises a group of diseases characterized by accumulation of mast cells on the skin, with the possibility of affecting other systems. Symptoms can be exclusively cutaneous, associated with symptoms of the organs involved or even systemic, due to the release of chemical mediators. Three cases of systemic mastocytosis are described, each case presenting different manifestations of the disorder. Description: the first report is about a patient with urticaria pigmentosa that presented persistent lesions until puberty when systemic manifestations initiated with lymphoreticular involvement, splenomegaly and bone marrow infiltration. In the second case, the child had bullous mastocytosis associated with gastrointestinal symptoms, whose investigation showed mast cell infiltration in the intestinal wall. The third patient presented an early and extensive cutaneous manifestation of mastocytosis, with a dramatic evolution to shock and posterior death.Discussion: clinical aspects, treatment and prognosis of such forms of the disease are discussed.
Keywords : mastocytosis; urticaria pigmentosa.