Diffuse cutaneous mastocytosis is a rare disease caused by a disturbance in the immune reaction, where mast cells (defense cells of the body) accumulate in the skin tissues or other tissues of the body, mainly in the bone marrow and gastrointestinal tract.
Mastocytosis can be limited to the skin or can involve other organs, such as the stomach, intestines, liver, spleen, lymph nodes and bones. Although rare, it can be associated with severe blood disorders, such as acute leukemia, lymphoma, chronic neutropenia or some myeloproliferative disorder, mast cell leukemia and aggressive mastocytosis.
Mastocytosis symptoms
Symptoms of mastocytosis can be:
- Pigmented urticaria: small reddish-brown spots on the skin; Peptic ulcer; Vomiting in a jet; Chronic diarrhea; Abdominal pain. Feeling dizzy when standing up; Lips and numb fingers.
The diagnosis is confirmed by high levels of histamine or prostaglandin D2 in the 24-hour urine, collected shortly after the crisis and by the increase in mast cells in skin lesions (histology).
Treatment for mastocytosis
The treatment of mastocytosis is done with the ingestion of 2 types of antihistamines: histamine receptor blockers 1, the type used in the treatment of allergies, and histamine receptor blockers 2, the type used in the treatment of peptic ulcers. However, when mastocytosis is associated with a serious underlying disorder, treatment is much more complex.
Mastocytosis in children sometimes cures itself.
