Urticaria pigmentosa is the name given to a type of mastocytosis. Urticaria pigmentosa is a disease that produces skin lesions and deeply itching. It is caused by excessive numbers of inflammatory cells (mast cells) in the skin. Other forms include solitary mastocytoma (a single lesion) and systemic mastocytosis (involvement in organs other than the skin). Urticaria pigmentosa is the childhood form of mastocytosis. Mast cells are specialized cells of connective tissue that release substances such as histamine (a chemical important in the inflammatory process) and heparin (an anti-clotting agent) when the body’s alarm mechanism is set off. When mast cells cluster and multiply excessively (proliferate), histamine and heparin are released into the skin (mastocytosis).



The characteristic skin lesions of urticaria pigmentosa appear in these areas. Urticaria pigmentosa is an orphan disease, affecting fewer than 200000 people in the United States. Urticaria pigmentosa is most often seen in children, but it can occur in adults as well. Rubbing of a lesion produces a rapid wheal (a hive-like bump). Younger children may develop a fluid-filled blister over a lesion if it is scratched. A large histamine release from these extra mast cells may cause flushing, headache, diarrhea, a rapid heartbeat (tachycardia), and even fainting. Most common symptoms of urticaria pigmentosa is red or brown spots are often seen on the skin, typically around the chest and forehead. Symptoms can range from very mild (flushing, hives, no treatment needed) to life-threatening (vascular collapse).

Most treatments for mastocytosis can be used to treat urticaria pigmentosa. Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis. The use of antihistamines is the most common treatment. To keep the disease from spreading these will help fight off the itching and flushing. Albuterol and other beta-2 agonists open airways that can constrict in the presence of histamine. Patients with urticaria pigmentosa should avoid aspirin, codeine, opiates, procaine, alcohol. polymyxin B, hot baths, and vigorous rubbing after bathing and showering. Photochemotherapy form of ultraviolet radiation is the most effective treatment for adults with urticaria pigmentosa. Two or three treatments each week are required for several months.

Urticaria Pigmentosa Treatment and Prevention Tips

1. Antihistamines may relieve symptoms such as itching and flushing.

2. Disodium cromoglycate orally may be helpful in some cases.

3. Proton pump inhibitors help reduce production of gastric acid.

4. Corticosteroids can be used topically reduce inflammation associated with mastocytosis.

5. Avoid aspirin, codeine, opiates, procaine, alcohol. polymyxin B, hot baths.

6. Potent steroid creams applied for several months under occlusion can reduce itching and unsightliness.