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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cutaneous mastocytosis


Other Names for this Disease

  • Diffuse cutaneous mastocytosis
  • Mastocytoma
  • Telangiectasia macularis eruptiva perstans
  • Urticaria pigmentosa
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Overview

What is cutaneous mastocytosis?

How might cutaneous mastocytosis be treated?

What is cutaneous mastocytosis?

Cutaneous mastocytosis is a condition caused by the accumulation of mast cells in the skin. Mast cells contain substances such as histamine that regulate allergic reactions. This condition is usually diagnosed in children and typically resolves by puberty. Treatment is generally based on a person's symptoms, but it may include antihistamines to prevent the effect of mast cell histamine.[1]

There are several different types of cutaneous mastocytosis. Urticaria pigmentosa is the most common form and is characterized by brown patches on the skin where mast cells have accumulated. Diffuse cutaneous mastocytosis is a very rare form of the condition that presents at birth with skin that is thickened and easily blistered. A mastocytoma is raised nodule that is also usually seen in infancy. Another rare form that occurs in adults is called telangiectasia macularis eruptiva perstans (TMEP).[2]
Last updated: 10/8/2012

How might cutaneous mastocytosis be treated?

Therapy for cutaneous mastocytosis aims to relieve symptoms.  Medications such as antihistamines and disodium cromoglycate (cromolyn) are available to target most skin and stomach-related symptoms. [3]

Other medications may be recommended for symptoms of more severe and unusual forms of cutaneous mastocytosis.  Corticosteroid therapy, both applied to the skin (topical) and by injection, as well as aspirin may be helpful for some individuals whose symptoms don't improve with antihistamines. Some severe cases of cutaneous mastocytosis may respond to ultraviolet light therapy such as psoralen plus UV-A (PUVA). These therapies must be carefully administered by a healthcare professional due to potentially severe reactions.  [3]

Therapies that stabilize the body's immune system (immune modulators) are currently being developed. [3]
Last updated: 10/8/2012

References
  1. Mastocytosis. National Institute of Allergy and Infectious Diseases. January 2011; http://www.niaid.nih.gov/topics/mastocytosis/Pages/Default.aspx. Accessed 5/15/2013.
  2. All About Mastocytosis. The Mastocytosis Society, Inc. 2011; http://tmsforacure.org/patients/mastocytosis_explained_2.php. Accessed 10/5/2012.
  3. Hogan D, Mastrodomenico CM. Mastocytosis: Treatment & Medication. Medscape Reference. June 2012; http://emedicine.medscape.com/article/1057932-treatment. Accessed 10/8/2012.


Other Names for this Disease
  • Diffuse cutaneous mastocytosis
  • Mastocytoma
  • Telangiectasia macularis eruptiva perstans
  • Urticaria pigmentosa
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.