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Urticaria Pigmentosa is the most common form of cutaneous mastocytosis. It is a rare disease. It is mainly caused by excessive amounts of mast cells in the skin. Urticaria pigmentosa is most often seen in children, but it can occur in adults as well. The condition occurs occurring primarily in children and clears spontaneously with adolescence. Urticaria pigmentosa is generally benign and is usually self-limited.


The exact cause of the disease is not known, although some cases may be inherited. This disease entry is based upon medical information available through the date at the end of the topic. Urticaria pigmentosa is one of several forms of mastocytosis, which is caused by excessive numbers of inflammatory cells (mast cells) in the skin. Here are the lists of some causes:

  • excessive numbers of inflammatory cells (mast cells) in the skin
  • systemic mastocytosis (involvement in organs other than the skin)
  • headache
  • diarrhea
  • a rapid heartbeat
  • Even fainting


It is an uncommon rash which usually affects the arms, necks, legs and trunks of children and young adults. The rashes are made up of reddish brown spots that turn into hives when they are rubbed or scratched. After the rash consists of reddish-brown spots it than turn into hives when they are rubbed hard or scratched. Sometimes the spots will blister. Mast cells are normally widely distributed over the skin. They contain granules that contain histamine and other chemicals. When the mast cell is disturbed, these chemicals are released into the surrounding skin.


There isno satisfactory treatment for urticaria pigmentosa. However, treatments are possible. Most treatments for mastocytosis can be used to treat Urticaria Pigmentosa.

Potent steroid creams applied for several months under occlusion can reduce itching and unsightliness, but the patches tend to recur within a few months.

Antihistamines may relieve some of the histamine-induced symptoms such as itching and flushing. Discuss the choice of antihistamine with your child's health care provider. With more severe and unusual forms of urticaria pigmentosa, systemic symptoms may need to be controlled with other medications.

Bee stings may also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor may advise you to carry an EpiPen to be used in case of a bee sting.

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