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Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations

Mariana C Castells, MD, PhD
Cem Akin, MD, PhD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD


Mastocytosis refers to a group of disorders characterized by excessive mast cell accumulation in one or multiple tissues. Mastocytosis is subdivided into two groups of disorders [1,2]:

Cutaneous mastocytosis (CM) describes forms of mastocytosis that are limited to the skin.

Systemic mastocytosis (SM) describes forms of mastocytosis in which pathologic mast cells infiltrate multiple extracutaneous organs, with or without skin involvement.

The epidemiology, pathogenesis, classification, and clinical manifestations of cutaneous and systemic mastocytosis will be reviewed here. The evaluation, diagnosis, treatment, and prognosis of mastocytosis are discussed separately. (See "Mastocytosis (cutaneous and systemic): Evaluation and diagnosis in adults" and "Systemic mastocytosis: Management and prognosis".)


Mastocytosis, in all its forms, is a rare disorder. The exact incidence is unknown. Mastocytosis affects males and females in equal ratios, although there is a slight male predominance in childhood and a slight female predominance in adulthood [3].

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Literature review current through: Nov 2017. | This topic last updated: Dec 05, 2016.
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