ABSTRACT

Background:

Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in body tissues. It usually presents in the skin, but may affect other tissues, especially the bone marrow, liver, spleen, and gastrointestinal tract. Cutaneous mastocytosis may be associated with both local and systemic symptoms, including flushing, blistering, pruritus, shortness of breath, asthma exacerbation, hypotension, and gastrointestinal upset, including acid reflux, peptic ulcer, or diarrhea. Symptomatic treatment is used in cutaneous mastocytosis. The majority of pediatric patients experience spontaneous remission of mastocytosis. This article presents a current overview for a better understanding of the symptoms associated with mastocytosis, to describe recent advances in its pathophysiology and treatment.