• J Chemli, S Krid, A Tfefha, S Abroug, and A Harbi.
• Service de pédiatrie, CHU Sahloul, 4000 Sousse, Tunisie. jalel_chemli@yahoo.fr
• Arch Pediatr. 2003 Oct 1; 10 (10): 898-902.

UnlabelledSystemic mastocytosis is rare in children and is characterized by an abnormal proliferation and infiltration of mast cells in different tissues.Case ReportWe report a case of systemic mastocytosis presenting cutaneous symptoms during the neonatal period. Later evolution was characterized by systemic manifestations consisting of recurrent respiratory infections with wheezing and a digestive involvement that included abdominal pain, hepatosplenomegaly and a nodular, hemorrhagic infiltrate in a low esophagus. The diagnosis was confirmed by histology and biology, notably increased histamine concentrations in blood and urines. Improvement of the respiratory and digestive symptoms was obtained with treatment by histamine H1 and H2 receptors antagonists.ConclusionRespiratory manifestations and nodular infiltration of the digestive tract are rare in systemic mastocytosis. The prognosis is conditioned by complications such as malignancy and the persistence of the disease till the adult age.

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