Mast cells, connective tissue cells, play an eminent role in defending tissue, including the skin and select organ tissue, from disease. Mastocytosis is a relatively rare disorder affecting approximately less than 1% of births in the United States. Mastocytosis is caused by the proliferation or presence of too many mast cells in children and adults. There are two types of mastocytosis. Cutaneous mastocytosis, also referred to as urticaria pigmentosa, first described circa 1869, occurs when mast cells enter or get into the skin and begin proliferation. Systemic mastocytosis, described circa 1933, refers to the proliferation of mast cells in organ tissue. Systemic mastocytosis may affect organs, including the liver, small intestine, and spleen.
Variety of rare disorders in which there is an abnormal proliferation of mast cells throughout the body and skin. Stroking or firm touching of the skin causes the mast cells to release histamine and other substances, leading to hives and itching. Organ infiltration may result in life threatening problems, such as respiratory or cardiac dysfunction.
A rare condition in which the primary abnormality is of mast cells. 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.
A general term for a variety of rare disorders in which there is proliferation of excessive numbers of normal mast cells systemically or in the skin. Lesions present on the skin are termed urticaria pigmentosa. Firm stroking of the skin lesion will cause the area to become raised and pruritic with surrounding erythema; this is Darier’s sign.
A rare disorder characterized by itchy, uneven, yellow, or orange-brown growths that emerge on the skin, often found on the torso, is referred to as mastocytosis. This condition can also impact internal organs like the liver, spleen, and intestines, potentially giving rise to symptoms like diarrhea, vomiting, and fainting. In extremely rare cases, it can lead to anaphylactic shock, an intense and potentially fatal allergic reaction.
Mastocytosis typically initiates within the initial year of life and tends to subside by adolescence. Symptoms can often be alleviated with the use of antihistamine medications.