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Genetic and Rare Diseases Information Center (GARD)

Cutaneous mastocytosis

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.


Cutaneous mastocytosis is a form of mastocytosis that primarily affects the skin. There are three main forms of the condition: maculopapular cutaneous mastocytosis (also called urticaria pigmentosa), solitary cutaneous mastocytoma, and diffuse cutaneous mastocytosis. There is also an exteremely rare form called telangiectasia macularis eruptiva perstans. The signs, symptoms and severity of the condition vary by subtype.[1][2][3] Cutaneous mastocytosis is usually caused by changes (mutations) in the KIT gene. Most cases are caused by somatic mutations which are not inherited or passed on to the next generation.[1] However, it can rarely affect more than one family member and be inherited in an autosomal dominant manner.[4] Treatment is usually symptomatic and may include oral antihistamines, topical steroids, and/or photochemotherapy.[5][1]

Last updated: 2/23/2015


  1. Mastocytosis. DermNet NZ. September 2014;
  2. Mastocytosis (cutaneous and systemic): Epidemiology, pathogenesis, and clinical manifestations. UpToDate. September 2014; Accessed 2/22/2015.
  3. Daniel J Hogan, MD. Mastocytosis. Medscape Reference. February 2015;
  4. Fett NM, Teng J, Longley BJ. Familial urticaria pigmentosa: report of a family and review of the role of KIT mutations. Am J Dermatopathol. February 2013; 35(1):113-116.
  5. Mariana C Castells, MD, PhD; Cem Akin, MD, PhD. Treatment and prognosis of cutaneous mastocytosis. UpToDate. October 2014; Accessed 2/23/2015.
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information on maculopapular cutaneous mastocytosis and mastocytoma, which are two specific types of cutaneous mastocytosis.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on Cutaneous mastocytosis.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Cutaneous mastocytosis. Click on the link to view a sample search on this topic.
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.