Mastocytosis in the skin in children and adults

Background Mastocytosis is a rare disease of bone marrow-derived hematopoietic progenitor cells, which is characterized by abnormal growth and excessive accumulation of mast cells in various tissues. Cutaneous Mastocytosis is a skin limited disease, whereas Systemic Mastocytosis usually involves bone marrow, spleen, liver, lymph nodes and gastrointestinal tract and may present with or without skin involvement. The skin is one of the most commonly affected organ both in children and adults. Mastocytosis in the skin (MIS) is defined by a typical exanthema and monomorphic mast cell infiltrate. MIS is an initial diagnosis which requires further diagnostic steps leading to the final diagnosis of Cutaneous Mastocytosis or Systemic Mastocytosis.