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Genetic and Rare Diseases Information Center (GARD)

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Cutaneous mastocytosis


Other Names for this Disease
  • Mastocytoma
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Your Question

What medications and/or treatments can I use for this condition? The itching is driving me crazy. At the moment I take anti-histamines, would PUVA treatments help? Will this condition ever leave?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might cutaneous mastocytosis be treated?

Although there is currently no cure for cutaneous mastocytosis, treatments are available to manage the symptoms of the condition. In general, it is recommended that affected people avoid things that trigger or worsen their symptoms when possible. Certain medications such as oral antihistamines and topical steroids are often prescribed to relieve symptoms. Affected adults may also undergo photochemotherapy which can help alleviate itching and improve the appearance of the patches; however, the condition is likely to recur within six to twelve months of the last treatment. People at risk for anaphylactic shock and/or their caregivers should be trained in how to recognize and treat this life-threatening reaction and should carry an epinephrine autoinjector at all times.[1][2][3]
Last updated: 2/23/2015

What is the long-term outlook for people with cutaneous mastocytosis?

The long-term outlook (prognosis) for people affected by cutaneous mastocytosis varies depending on the age at diagnosis. The prognosis is typically excellent in children who develop symptoms before age 2 as approximately 80% of these cases resolve before puberty. The remaining cases may persist as cutaneous mastocytosis or progress to systemic disease. Risk factors for progression to systemic mastocytosis include later onset of the skin symptoms (after age 2), persistence of skin symptoms past adolescence, and abnormal blood counts. In very rare cases, the condition may progress to aggressive forms of mast cell disease such as mast cell sarcoma (solid tumor of cancerous mast cells) or mast cell leukemia (rare disease of circulating cancerous mast cells).[3][4]

Cutaneous mastocytosis that develops after age 2 or in adults generally does not resolve. Approximately 90% of affected adults also have evidence of systemic mastocytosis at the time of diagnosis. Later onset cases also have a higher risk of malignant (cancerous) forms of mast cell disease.[3][4]
Last updated: 2/24/2015

References
Other Names for this Disease
  • Mastocytoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.