National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Maculopapular cutaneous mastocytosis

Not a rare disease Not a rare disease
Other Names:
Urticaria pigmentosa
This disease is grouped under:
Maculopapular cutaneous mastocytosis (also known as urticaria pigmentosa) is a common form of cutaneous mastocytosis characterized by brown patches or freckles on the skin that result from an abnormal collection of mast cells.[1][2] The skin may be very itchy and rubbing may lead to hive-like bumps or fluid-filled blisters.[3] The condition is most common in children, but adults can also be affected.[1][2][3] Treatment may include antihistamines, topical steroids, and/or phototherapy. Over time, the mastocytosis in younger patients usually becomes less itchy and the skin patches tend to improve. In adults, systemic mastocytosis may develop.[3] 
Last updated: 9/22/2015

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cutaneous mastocytosis 0200151
Hypermelanotic macule
Hyperpigmented spots
0001034
Papule 0200034
Pruritus
Itching
Itchy skin
Skin itching
[ more ] 		0000989
Skin plaque 0200035
30%-79% of people have these symptoms
Cardiac arrest
Heart stops beating
0001695
Diarrhea
Watery stool
0002014
Dyspnea
Trouble breathing
0002094
Headache
Headaches
0002315
Nausea and vomiting 0002017
Rhinitis
Nasal inflammation
0012384
5%-29% of people have these symptoms
Skin nodule 0200036
Telangiectasia of the skin 0100585
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
Edema
Fluid retention
Water retention
[ more ] 		0000969
Erythema 0010783
Telangiectasia macularis eruptiva perstans 0007583
Urticaria
Hives
0001025
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Last updated: 7/1/2020
Maculopapular cutaneous mastocytosis occurs when there are too many inflammatory cells (called mast cells) in the skin.[3] The underlying reason for this excess of mast cells is not fully understood. While most cases appear to be random (occur sporadically for no apparent reason), familial cases have been reported. These familial cases are thought to be inherited in an autosomal dominant manner and may be caused by mutations in the KIT gene.[2][4]
Last updated: 9/24/2015
Maculopapular cutaneous mastocytosis is generally not serious and many cases do not require treatment. For those cases that do, oral antihistamines, topical steroids, and a photochemotherapy called PUVA may be used.[1] These treatments are aimed at reducing the symptoms of the condition and making patients more comfortable.
Last updated: 9/24/2015
The prognosis for children with maculopapular cutaneous mastocytosis is good. Up to 50% of cases improve and 30% of cases resolve completely by adolescence.[3][2]
Last updated: 9/24/2015

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Maculopapular cutaneous mastocytosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • 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 Manual 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.
  • The Mastocytosis Society has developed a Pediatric Mastocytosis Fact Sheet which includes information about maculopapular cutaneous mastocytosis.
  • The Mastocytosis Society provides information about mast cell diseases, including Maculopapular cutaneous mastocytosis.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • 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 Maculopapular cutaneous mastocytosis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • What causes maculopapular cutaneous mastocytosis? Can it be treated? Can it be cured? See answer


  1. Yung A. Maculopapular cutaneous mastocytosis. DermNet NZ. March 2014; http://www.dermnetnz.org/colour/urticaria-pigmentosa.html. Accessed 9/22/2015.
  2. Oranje A. Maculopapular cutaneous mastocytosis. Orphanet. June 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=79457. Accessed 9/22/2015.
  3. Moskowitz RJ, Zieve D. Urticaria pigmentosa. MedlinePlus. November 20, 2014; https://www.nlm.nih.gov/medlineplus/ency/article/001466.htm. Accessed 9/22/2015.
  4. Urticaria Pigmentosa. National Organization for Rare Disorders (NORD). 2003; https://rarediseases.org/rare-diseases/urticaria-pigmentosa/. Accessed 9/24/2015.