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Urticaria Pigmentosa (Mastocytosis)

Urticaria pigmentosa is a skin disease, which is a form of urticaria as well as mastocytosis, in which you find brown, intensely itching patches on skin. On rubbing the spots, one gets hives or urticarial patches.

It is one of the forms of mastocytosis. For understand mastocytosis, we need to understand a bit about mast cells. Mast cells are normal cells found in skin all over the body. Mast cells contain certain granules which when activated or stimulated, lead to release of histamine and other such chemicals, leading to the condition called mastocytosis. You may say, mast cells are inflammatory cells.

Incidence:

Urticaria pigmentosa is most commonly seen in infants and children but can also be found in adults.

Causes:

The exact cause of this uncommon disease is unknown but recent research suggests genetic change in a protein (called c-kit) on the surface of mast cells may result in the abnormal proliferation of these cells.

Variety of factors can cause or worsen the symptoms of urticaria pigmentosa:

  • Physical stimuli such as heat, friction, and excessive exercise
  • Bacterial toxins
  • Venom
  • Eye drops containing dextran
  • Alcohol
  • Morphine
  • Emotional stress

Symptoms:

  • Brownish lesions on skin are seen typically around chest and forehead.
  • Rubbing the skin sore causes hive like bump.
  • A fluid filled blister may form if it is scratched, especially seen in younger children.
  • Face may become flushed.
  • In severe cases diarrhea, headache and palpitation may be seen.

Darier's sign:

A typical presentation called Darier’s sign is observed in Urticaria pigmentosa. By rubbing one of the brown patches the rubbed area becomes reddened, swollen and itchy. This confirms the presence of mastocytosis

Something about mast cells:

Mast cells are normally widely distributed in 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. The chemicals make the blood vessels leaky, resulting in localized itching, swelling and redness.

Diagnosis and Investigations:

  • The disease is most often diagnosed clinically
  • Doctors can confirm the presence of mast cells by rubbing the skin. If hives appear, it most likely signifies the presence of urticaria pigmentosa.
  • Skin biopsy shows an increase in mast cells and confirms the diagnosis.

If there are any symptoms suggesting internal involvement the following tests may be helpful

  • X-rays of skull and long bones may show areas of bone thinning, or a diffuse ground glass appearance
  • Urinary histamine levels or 1,4-methylimidazole acetic acid may be elevated

Prognosis:

  • It goes away on its own till the time of puberty in 50% of the cases but medication is required as it is a troublesome disease.
  • In adults it may develop into more serious condition called systemic mastocytosis.

Treatment:

  • a. Conventional treatment for Urticaria pigmentosa: Urticaria pigmentosa is hard to treat with the conventional medicine.
    • Oral antihistamines are useful because they reduce the mast cell's ability to react to histamine.
    • Disodium cromoglicate orally may be helpful in some cases as they act as mast cell stabilizers.
    • Topical steroids have limited role.
    • Photo chemotherapy for longer periods may be helpful to some extent.
  • b. Homeopathic treatment for Urticaria pigmentosa:
    • Homeopathy works effectively for urticaria pigmentosa. Homeopathy evaluation takes into account genetic, immunological and allergic factors in particular while treating this conditions. Results are positive and encouraging. Strongly recommended.
    • The line of homeopathic treatment is largely constitutional therapy. Some of the commonly indicated homeopathic medicines include Natrum muriaticum, Sulphur, Thuja occidentalis, Silica, Apis melifica, Arsenic iodum, etc.
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