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Cutaneous mastocytosis in children Volume 19, issue 3, Juillet-Août-Septembre 2016

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Authors
1 Hôpital Errazi, CHU de Med VI, Service de dermatologie, CHU Med VI, Hôpital Errazi, 40000, Marrakech ; Faculté de Médecine et de Pharmacie, Université Caddi Ayyad, Marrakech, Maroc
2 Laboratoire « ZOHOR » d’anatomie et cytologie pathologiques, Marrakech, Maroc
* Tirés à part

Mastocytoses are rare diseases, which are most often sporadic and characterised by the proliferation and abnormal accumulation of mast cells in cutaneous tissue (cutaneous mastocytosis [CM]) and extracutaneous organs such as the bone marrow, liver, spleen, and lymph nodes ; systemic mastocytosis (SM). Nearly two thirds of cases of mastocytosis affect children, most often in the pure cutaneous form. It is possible for the disease to regress at puberty. In adults, mastocytosis is systemic in nearly a third of cases, with a potential chronic evolution of the disease and the possibility of aggressive forms which are often associated with haematological damage, which makes the prognosis of the disease severe. Cutaneous manifestations of mastocytosis are partly linked to the accumulation of mast cells in the skin and partly to the mediator effect of the mast cells. Diagnosis is based on histopathological examination which has to be guided by clinical examination and coupled with an immunohistochemical examination in difficult cases. The clinical forms of CM are dominated by urticaria pigmentosa and nodular-papular mastocytosis. The treatment of CM in children is mostly symptomatic and transient because of the spontaneous regression of the disease in half of the cases during puberty. Treatment is based on the elimination of all mast cell degranulation factors, antihistamine H1 with or without antihistamine H2, topical corticosteroids, PUVA therapy, local care in the event of bullous lesions, and surgical excision of mastocytomas.