Mastocytosis, Vascular, Muscular and Fibrohistiocytic Tumors

Mastocytosis is a very heterogeneous disease. In cutaneous mastocytosis, the abnormal growth of mast cells is limited to a cutaneous lesion while in systemic mastocytosis at least one extracutaneous organ is involved. An updated consensus classification for mastocytosis was proposed in 2001 (Table 3.6.1) [4]. In cutaneous mastocytosis, normal-appearing mast cells are increased predominantly around blood vessels and skin appendages in the papillary dermis. The diagnosis of systemic mastocytosis is made by multifocal compact mast cell infiltrations consisting at least 15 cells that are detected in bone marrow or other extracutaneous organs [4].