Definition:
Systemic mastocytosis, often called systemic mast cell disease (SMCD), is marked by mast cell infiltration of extracutaneous organs, which is in contrast to cutaneous mast cell disorders, which involve only the skin.
Diagnosis:
Doctors can diagnose urticaria pigmentosa (cutaneous mastocytosis, see below) by observing the characteristic lesions that are dark-brown and fixed. A small skin sample (biopsy) may aid in confirming the diagnosis.
Treatment:
There is currently no cure for mastocytosis. However, there are a number of medicines to help treat the symptoms of mastocytosis, such as: antihistamines, leukotriene antagonists, mast cell stabilizers, proton pump inhibitors, and epinephrine.
Symptoms and Signs:
Some symptoms include: itching, abdominal cramping; anaphylaxis; skin lesions; abdominal discomfort; episodes of very low blood pressure (including shock) and faintness; bone or muscle pain; and nausea and vomiting.
Causes:
This disorder is caused by the presence of too many mast cells (mastocytes) and CD34+ mast cell precursors in a person's body.
