期刊
JOURNAL OF CLINICAL MEDICINE
卷 10, 期 7, 页码 -出版社
MDPI
DOI: 10.3390/jcm10071474
关键词
mastocytosis; cutaneous mastocytosis; systemic mastocytosis; children; antihistamines; PUVA; cromolyn sodium; urticaria pigmentosa
Mastocytosis is a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. It is classified into cutaneous mastocytosis and systemic mastocytosis. In children, cutaneous mastocytosis is the most frequent form.
Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. Broadly, M is classified into two categories: Cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In children, CM is the most frequent form. Unfortunately, pathogenesis is still unclear. It is thought that genetic factors are involved, but further studies are necessary. As for features of CM, the lesions differ in clinical forms. The most important fact is evaluating a pediatric patient with CM. It must comprise laboratory exams (with baseline dosing of total serum tryptase), a skin biopsy (with a pathological exam and, if the diagnosis is unclear, immunohistochemical tests), and a complete clinical evaluation. It is also defining to distinguish between CM and other diseases with cutaneous involvement. As for the management of CM in children, the first intervention implies eliminating trigger factors. The available cures are oral H1 and/or H2 antihistamines, oral cromolyn sodium, oral methoxypsoralen therapy with long-wave psoralen plus ultraviolet A radiation, potent dermatocorticoid, and calcineurin inhibitors. In children, the prognosis of CM is excellent, especially if the disease's onset is in the first or second years of life.
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