Management of Cutaneous Mastocytosis
Abstract
The mastocytoses are a group of mast cell proliferation disorders that can result in both cutaneous and systemic symptoms. Mastocytosis can initiate at birth or may appear at any point thereafter, even in late adulthood. Mastocytosis with a childhood onset is described as beginning prior to puberty. Most frequently, adult-onset mastocytosis appears in the third or fourth decade of life. Mastocytosis affects individuals of all racial and gender identities. The majority of patients with mastocytosis have no family history of the condition; however, familial cases have been reported in the literature.
The World Health Organization (WHO) has classified mastocytosis into a number of categories:
1) Cutaneous mastocytosis (CM); 2) Indolent systemic mastocytosis (ISM); 3) Systemic mastocytosis with a nonmast-cell clonal hematologic disorder (SM-AHNMD); 4) Aggressive systemic mastocytosis (ASM); 5) Mast cell leukaemia (MCL); 6) Mast cell sarcoma; and 7) Extracutaneous mastocytoma. The most frequent site of organ involvement in individuals with mastocytosis is the skin and this will be the focus of this article.
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