Dermatitis Herpetiformis

Dermatitis Herpetiformis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Dermatitis Herpetiformis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Brocq-Duhring Disease
  • Duhring Disease
  • Dermatitis Multiformis
  • DH

Disorder Subdivisions

  • None

General Discussion

Dermatitis herpetiformis, also known as Duhring disease, is a rare, chronic, skin disorder characterized by the presence of groups of severely itchy (pruritic) blisters and raised skin lesions (papules). These are more common on the knees, elbows, buttocks and shoulder blades. The exact cause of this disease is not known although it is frequently associated with the inability to digest gluten (gluten sensitive enteropathy [GSE] or celiac sprue).

Symptoms

The slow onset of the symptoms of dermatitis herpetiformis usually begins during adulthood, but children can also be affected. Symptoms include the appearance on the skin of blisters, fluid-filled sores, red lesions that resemble hives, and/or well-defined, raised skin lesions (papules). The skin typically becomes intensely itchy and red. Itching and burning may be almost intolerable and the need to scratch may become irresistible. Skin lesions develop on both sides of the body, especially on the head, elbows, knees, lower back, and/or buttocks. Frequently people with dermatitis herpetiformis develop blisters and papules on the face and neck. Approximately fifty percent of people with Duhring disease have dark discolorations (pigmentation) in the areas where skin lesions are present.



Approximately seventy-five percent of people with dermatitis herpetiformis also have an increased sensitivity to gluten. Gluten is a protein that is present in wheat, oats, rye, barley, and millet. The symptoms associated with the loss of the ability to properly digest gluten (gluten-sensitive enteropathy) are similar to those of celiac disease. (For more information on celiac disease, see the Related Disorders section of this report.)

Causes

The exact cause of dermatitis herpetiformis is not fully understood. Several studies have suggested that there may be a genetic predisposition to this disease. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease. Approximately ninety percent of people with this disease are positive for HLA-B8 gene, a marker that may indicate a predisposition to this disease.



Abnormalities of the immune system, especially the presence of IgA (immunoglobulin A) in skin lesions, are common in patients with dermatitis herpetiformis. Particular drugs, such as iodide and other halides can cause a flare-up of symptoms. The exact role of gluten as a cause of this disease is not understood.



Recently, the presence of epidermal transglutaminase has been detected in the skin of patients with dermatitis herpetiformis. Epidermal transglutaminase is very closely akin to tissue transglutaminase, the enzyme characteristic of celiac disease. Research continues to determine whether this finding may help to explain what causes dermatitis herpetiformis.

Affected Populations

Dermatitis herpetiformis affects more males than females by a ratio of 3:2. It is estimated that this disorder affects as many as 39 cases per 100,000. Although the symptoms may begin at any age, this disease usually begins during middle adult life. It is very rare in children.

Standard Therapies

Diagnosis

The diagnosis of dermatitis herpetiformis may be made by direct immunofluorescence testing on samples of skin tissue. This test is designed to demonstrate the presence of IgA, a protein antibody (immunoglobulin), not normally in skin tissue.



Treatment

People with this disease are typically treated with the drug dapsone. This drug usually relieves the symptoms and improves the skin rash within 1 or 2 days. The urgent need to scratch usually subsides within 1 to 3 days. Since one of the most common side effects of dapsone is the destruction of red blood cells (hemolysis), people taking this medication must have periodic blood tests to monitor for any changes in their blood counts.



The application of a topical corticosteroid cream to affected areas on the skin may help to relieve itching in some people with this disease. However, the administration of oral nonsteroidal antiinflammatory drugs (NSAIDS) usually aggravates the symptoms of dermatitis herpetiformis.



Some people with dermatitis herpetiformis who also have gluten sensitive enteropathy may be able to discontinue drug therapy if they follow a strict gluten-free diet for at least 6 to 12 months. Gluten is found in all grains except corn and rice. Special gluten-free foods are commercially available.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Researchers are exploring the relationship between the skin and the function of the digestive system in people with dermatitis herpetiformis and other similar dermatological diseases. For more information, please contact:



Russell P. Hall, III, M.D.

Box 3135

Duke University Medical Center

Durham, NC 27710

(919) 684-3110

References

TEXTBOOKS

Hall RP III. Dermatitis Herpetiformis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:103.



Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:830-31.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1217-18.



Champion RH, Burton JL, Ebling FJG. Eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992:1658-62.



REVIEW ARTICLES

Clarke P. Why am I so itchy? Aust Fam Physician. 2004;33:489-94.



Duggan JM. Coeliac disease: the great imitator. Med J Aust. 2004;180:524-26.



Karpati S. Dermatitis herpetiformis: close to unraveling a disease. J Dermatol Sci. 2004;34:83-90.



Collin P. Reunala T. Recognition and management of cutaneous manifestations of celiac disease: a guide for dermatologists. Am J Clin Dermatol. 2003;4:13-20.



Fry L. Dermatitis herpetiformis: problems, progress and prospects. Eur J Dermatol. 2002;12:523-31.



Bickle K, Roark TR, Hsu S. Autoimmune bullous dermatoses: a review. Am Fam Physician. 2002;65:1861-70.



FROM THE INTERNET

McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Dermatitis Herpetiformis, Familial. Entry Number; 601230: Last Edit Date; 11/18/2004.



Drayer J. Dermatitis herpetiformis. Medical Encyclopedia. MedlinePlus. Update date: 10/27/2003. 2pp.

www.nlm.nih.gov/medlineplus/ency/article/001480.htm



Fabbri P, Caproni M. Dermatitis herpetiformis. orphanet. Update: February 2005. 6pp.

www.orpha.net/data/patho/GB/uk-DermatitisHerpetiformis.pdf



Fry L. What Is DH? The Dermatitis Herpetiformis Online Community. nd. 3pp.

www.dermatitisherpetiformis.org.uk/whatisdh.html



Dermatitis Herpetiformis. Gluten Intolerance Group. Website last Updated July 21, 2005. 2pp.

www.gluten.net/dh.html

Resources

Gluten Intolerance Group of North America

31214 124th Ave SE

Auburn, WA 98092

Tel: (253)833-6655

Fax: (253)833-6675

Email: info@gluten.net

Internet: http://www.gluten.net



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



National Foundation for Celiac Awareness

P.O. Box 544

224 South Maple Street

Ambler, PA 19002

Tel: (215)325-1306

Fax: (215)643-1707

Email: info@celiaccentral.org

Internet: http://www.CeliacCentral.org



Australasian Blistering Diseases Foundation

St. George Hospital,

Department of Dermatology

Gray Street

Kogarah

Sydney, NSW 2217

Australia

Tel: 0291132088

Fax: 0291132886

Email: info@blisters.org.au

Internet: http://www.blisters.org.au/BDHome.html



Coeliac Society of Australia Inc.

Suite 1, 41 - 45 Pacific Highway

Waitara, NSW 2077

Australia

Tel: 0294875088

Fax: 0294875177

Tel: 1800458836

Email: info@coeliacsociety.com.au

Internet: http://www.coeliacsociety.com.au



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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