Mastocytosis is uncommon and is characterized by increased proliferation of mast cells. It is most commonly limited to the skin where it can be classified as isolated nodules (mastocytomas), diffuse multiple benign hyperpigmented macules or papules (urticaria pigmentosa), diffuse cutaneous involvement with boggy thickening of the skin (diffuse mastocytosis), and diffuse telangiectases (telangiectasia macularis eruptiva perstans).1 Mastocytosis is not normally a systemic disease but when it occurs it can be classified either as a benign form, in which skin, liver, spleen, gastrointestinal tract, and bones are infiltrated with mast cells, or a malignant form, characterized by tissue infiltration and a leukemic-like picture of the bone marrow and peripheral blood.2,3 The prognosis is generally favorable when the disease is localized to the skin in childhood but is variable with the onset in adult life and with systemic involvement.4
Systemic symptoms are common in all forms of mastocytosis and
Dantzig PI. Tetany, Malabsorption, and Mastocytosis. Arch Intern Med. 1975;135(11):1514–1518. doi:10.1001/archinte.1975.00330110104014
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