Mastocytosis

Abstract

Mastocytosis is a neoplastic disease involving mast cells (MC) and their CD34+ progenitors and consists of a heterogeneous group of diseases with a clinical spectrum ranging from localized mastocytoma to generalized forms such as mast cell leukemia. In 80% of mastocytosis, the skin is involved. Cutaneous mastocytosis (CM) has to be distinguished from systemic mastocytosis. CM is divided into maculopapular CM (= urticaria pigmentosa), diffuse cutaneous mastocytosis, teleangiectasia macularis eruptive perstans, and solitary mastocytoma of skin. All forms of mastocytosis can result in systemic symptoms induced by IgE-dependent or -independent degranulation of MC mediators (e.g., induced by hymenoptera stings or histamine liberating drugs). In this chapter, two cases of cutaneous and systemic mastocytosis are discussed.KeywordsCutaneous mastocytosisUrticaria pigmentosaTeleangiectasia macularis eruptive perstansSerum tryptaseMast cellsSystemic mastocytosisHistamineTryptase