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

Dermatitis herpetiformis


Other Names:
Duhring Brocq disease; Brocq-Duhring disease ; Duhring's disease; Duhring Brocq disease; Brocq-Duhring disease ; Duhring's disease; DH; Duhring-Brocq disease See More
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Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. The slow onset of symptoms usually begins during adulthood, but children can also be affected. Other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning. The exact cause of this disease is not known, but it is frequently associated with the inability to digest gluten. People with this disease are typically treated with the drug dapsone.[1][2]
Last updated: 10/29/2014

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
Abnormal blistering of the skin
Blistering, generalized
Blisters
[ more ] 		0008066
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ] 		0002960
Erythema 0010783
Macule
Flat, discolored area of skin
0012733
Malabsorption
Intestinal malabsorption
0002024
Microcytic anemia 0001935
Pruritus
Itching
Itchy skin
Skin itching
[ more ] 		0000989
Recurrent fractures
Increased fracture rate
Increased fractures
Multiple fractures
Multiple spontaneous fractures
Varying degree of multiple fractures
[ more ] 		0002757
Skin vesicle 0200037
Urticaria
Hives
0001025
30%-79% of people have these symptoms
Eczema 0000964
5%-29% of people have these symptoms
Bone pain 0002653
Edema
Fluid retention
Water retention
[ more ] 		0000969
Lichenification 0100725
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance 0000006
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Last updated: 7/1/2020
The antibiotic dapsone is extremely effective in treating this condition.[2] Symptomatic improvement may occur in as little as several hours after the first dose.[3] However, dapsone may cause serious side effects and requires regular monitoring by a physician. When this medication is used to relieve the symptoms of dermatitis herpetiformis, it should be taken in the smallest effective dose and for the shortest period possible.[4] In some cases, immunosuppressive medications may be used. These medications do not appear to be as effective.[2] A strict gluten-free diet is also recommended to help control the disease. Following this diet may eliminate the need for medications and prevent later complications.[2][3]
Last updated: 10/29/2014

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 Dermatitis herpetiformis . 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.

Patient Registry

  • The Autoimmune Registry supports research for Dermatitis herpetiformis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

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

  • 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 National Digestive Diseases Information Clearinghouse (NIDDK) offers information on this condition. Click on the link to view information on this topic.
  • 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.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • 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.
  • 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 Dermatitis herpetiformis . 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.

  1. Dermititis Herpetiformis. NORD. 2005; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/208/viewAbstract. Accessed 10/29/2014.
  2. Berman K, Zieve D. Dermatitis herpetiformis. MedlinePlus. May 15, 2013; http://www.nlm.nih.gov/medlineplus/ency/article/001480.htm. Accessed 10/29/2014.
  3. Miller JL. Dermititis Herpetiformia Treatment & Management. Medscape Reference. June 12, 2014; http://emedicine.medscape.com/article/1062640-treatment#showall. Accessed 10/29/2014.
  4. Dermatitis Herpetiformis. Gluten Intolerance Group. October 23, 2014; https://www.gluten.net/resources/dermatitis-herpetiformis/. Accessed 10/29/2014.