Mastocytoses

Czech version

Authors: Z. Plzáková; J. Štork
Authors‘ workplace: Dermatovenerologická klinika 1. LF UK a VFN, Praha přednosta prof. MUDr. J. Štork, CSc.
Published in: Čes-slov Derm, 93, 2018, No. 3, p. 91-100
Category: Reviews (Continuing Medical Education)

Overview

Mastocytosis is a group of diseases arising from the pathological increase of mast cells in tissues leading to local and/or systemic symptoms. The disease most commonly affects the skin with appearance of typical skin manifestations due to  an accumulation of mast cells, the mediator symptoms arise from the release of the mast cell granule content.

Diagnosis of skin manifestations of mastocytoses is clinical and histological. In adult mastocytosis, systemic involvement should be excluded by laboratory examination, imaging methods and bone marrow biopsy. In children, the systemic form is rare and the skin manifestations mostly disappear to puberty. Systemic disease in children is considered if symptoms persist after puberty, or severe mediatory symptoms or hepatosplenomegaly are present. The mast cell therapy is based on the severity of the symptoms. It includes local treatment, phototherapy, systemic symptoms are treated by antimediator remedies and immunosuppressive therapy, anti-receptor antibodies, kinase inhibitors, in aggressive forms by cytoreductive therapy. Equally important is prevention based on elimination of physical, food or drug triggers of degranulation of mastocytes.

Key words:

mastocytosis – mast cell mediators – skin mastocytosis – systemic mastocytosis – c-kit mutation – mediator release symptoms – prognosis – dianosis – therapy

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Labels

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Article was published in

Czech-Slovak Dermatology

2018 Issue 3