Systemic Mastocytosis

Excerpt

Mastocytosis is a constellation of disorders where there are excessive proliferation and accumulation of pathologic mast cells in the tissues. It can involve only skin, which is known as cutaneous mastocytosis, or it can involve extracutaneous tissues which is known as systemic mastocytosis. Cutaneous mastocytosis usually has a benign presentation. Systemic mastocytosis, in particular, is an aggressive form of the disorder characterized by the release of numerous vasoactive cell mediators due to excessive activity of mast cells, which can result in a wide variety of symptoms. The most frequent symptoms associated with the disease include pruritis, flushing, itching, diarrhea, and anaphylaxis.