Mastocytosis is characterized by large amounts of mast cells (MCs) accumulating in the body. Abnormal activation of MCs leads to accelerated growth and accumulation in the skin, bone marrow, and internal organs (liver, spleen, and gastrointestinal tract). Mastocytosis is divided into 7 categories based on World Health Organization classification. Two major types are cutaneous (CM) and systemic (SM) mastocytosis. There are several types of CM, including maculopapular (MPCM) and diffuse (DCM). SM also has several subtypes, including indolent and aggressive. These classifications are defined by the age of the patient at disease onset and histopathologic criteria (Table).
There are a variety of symptoms due to histamine, prostaglandins, leukotrienes, chemokines, and tryptase, which are released during MC activation. Skin manifestations range from mild pruritus to anaphylaxis, with 10% of pediatric patients developing anaphylaxis. However, in SM, symptoms may include flushing and pruritus, headache, vomiting, diarrhea, abdominal cramping, and hypotension....
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