Review article
https://doi.org/10.26800/LV-146-supl1-35
Mastocytosis in children
Slobodna Murat-Sušić
Anamaria Balić
Ivana Martinac Ciglar
Karmela Husar
Mihael Skerlev
Abstract
Mastocytosis are a heterogeneous group of diseases characterized by proliferation and accumulation of mastocytes in various tissues. Cutaneous mastocytosis is diagnosed if the accumulation of mastocytes is detected only in the skin. In cases of systemic mastocytosis the mastocytes accumulate in the bone marrow, gastrointestinal tract, lymph nodes, liver and/or spleen. Contrary to adults, in who systemic mastocytosis is the most common type of mastocytosis, in childhood the vast majority of patients are diagnosed with cutaneous forms of the disease. The type, distribution and extent of skin lesions, as well as symptoms, can vary from patient to patient. Symptoms are due to release of inflammatory mediators from mastocytes, most often itch, redness of
the skin and wheals. Regular follow-ups are necessary, while organomegaly, significantly elevated serum tryptase levels and/or detection of KIT mutation in peripheral blood help in decision if and when to perform bone marrow biopsy. Treatment is aimed toward avoidance of triggers, and alleviation of symptoms caused by mast cell mediators, usually H1 and H2 blockers, as well as topical corticosteroids and pimecrolimus application.
Keywords
Hrčak ID:
316049
URI
Publication date:
11.4.2024.
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