Mastocytosis - Symptoms & Treatment

Mastocytosis is a disorder in both children and adults. There are three main forms of mastocytosis. In a rare form, mast cells accumulate as a single mass in the skin. Typically, a mastocytoma develops before age 6 months. In a form called urticaria pigmentosa, mast cells accumulate in many areas of the skin, forming small reddish brown spots or bumps. It is a group of disorders characterized by proliferation of mast cells and infiltration of the skin, other organs, or both. Pathology results mainly from release of mast cell mediators, including histamine, heparin, leukotrienes, and various inflammatory cytokines. Mast cells seem to have other roles as well. Because they gather together around wounds, mast cells may play a part in wound healing. For example, the typical itching felt around a healing scab may be caused by histamine released by mast cells. The presence of too many mast cells, or mastocytosis, can occur in two forms-cutaneous and systemic. The most common cutaneous form is also called urticaria pigmentosa, which occurs when mast cells infiltrate the skin.

Mastocytosis can come on at any age, but the disease is different when adults get it. Children with mastocytosis usually get it in the first year of life. A single mastocytoma does not cause symptoms. Rubbing or scratching the spots of urticaria pigmentosa may make the spots itch. Consuming hot beverages, spicy foods, or alcohol may also make itching worse. Rubbing or scratching the spots may result in hives and make the skin turn red. Flushing and widespread reactions, including anaphylactic reactions, may occur. Children with cutaneous forms require no additional treatment, because most cases resolve spontaneously. Adults with cutaneous forms may be treated with psoralen plus ultraviolet light or with topical corticosteroids once or bid. Mastocytoma usually involutes spontaneously and requires no treatment. The true incidence of either type of mastocytosis remains unknown, but mastocytosis generally is considered to be an "orphan disease." Topical cortisone creams and wet wraps have also been used.

Causes of Mastocytosis

Common causes and risk factor's of Mastocytosis:

• Blood disorders.
• Excessive number of mast cells.
• Cancer.
• Family history of mastocytosis.
• A mutation of c-kit.

Symptoms of Mastocytosis

Some sign and symptoms related to Mastocytosis are as follows:

• Abdominal cramping.
• Many pink or brown spots on the skin.
• Difficulty in breathing.
• Low blood pressure and faintness.
• Tummy pain.
• Nausea and vomiting.

Treatment of Mastocytosis

Common treatment of Mastocytosis:

• Orally administered cromolyn sodium can relieve diarrhea and abdominal pain.
• Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
• Malabsorption can be treated with steroids.
• Antidepressants are an important and often overlooked tool in the treatment of mastocytosis.