An unusual condition in which itchy, irregular, yellow or orangebrown swellings occur on the skin, most commonly on the trunk. Mastocytosis may also affect body organs, including the liver, spleen, and intestine, and it may cause symptoms such as diarrhoea, vomiting, and fainting. Very rarely, the condition leads to anaphylactic shock, which can be fatal. The condition usually begins in the 1st year of life and clears up by adolescence. Antihistamine drugs may be helpful in relieving symptoms of mastocytosis.
A rare condition in which the primary abnormality is of MAST CELLS – a type of cell responsible for the storage and release of agents such as HISTAMINE, important in allergic states. Patients may present with an urticarial rash (urticaria pigmentosa) but may have symptoms referable to any part of the body, related to collections of active mast cells in these areas.
n. a condition caused by *mast cell proliferation due to KIT *proto-oncogene mutations. It most commonly manifests in the skin but also affects other organs. Maculopapular variants were formerly known as urticaria pigmentosa. Systemic involvement is common in adult cases, with bone marrow examination essential for accurate diagnosis. It may be associated with haematological abnormalities, including leukaemias. Resolution is common in children with skin involvement only. Treatment includes antihistamines, mast-cell stabilizers (such as sodium *cromoglicate), and *tyrosine kinase inhibitors.
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