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Dermatitis Herpetiformis

Synonyms of Dermatitis Herpetiformis

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

Disorder Subdivisions

  • No subdivisions found.

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.

Related Disorders

Symptoms of the following disorders can be similar to those of dermatitis herpetiformis. Comparisons may be useful for a differential diagnosis:

Linear IgA disease (Linear IgA dermatosis) is a rare chronic skin disease characterized by the development of groups of itchy skin blisters and raised lesions (papules). It is not associated with the inability to digest gluten (gluten-sensitive enteropathy). Blisters, fluid-filled sores, and hives develop on the skin, especially on the arms, legs, lower back, and/or buttocks. The skin may become very red and extremely itchy. The exact cause of Linear IgA Disease is not known.

Bullous pemphigoid is a rare chronic skin disorder characterized by blistering sores on the skin. It occurs most frequently in the elderly population. The first symptom of Bullous Pemphigoid is usually redness of the skin that surrounds a skin lesion. Within weeks, thin-walled blisters with clear fluid centers (bullae) appear on the arms, legs, underarms (axilla), abdomen, and/or groin. If the blisters rupture, pain may be acute, but healing is usually rapid. (For more information on this disorder, choose "Bullous Pemphigoid" as your search term in the Rare Disease Database.)

Pemphigus is a group of rare autoimmune skin disorders characterized by the development of blisters on the outer layers of the skin (epidermis) and the mucous membranes of the mouth, nose, and/or genitals. The location and type of blister may vary according to the type of pemphigus. Symptoms may include soft blisters of the skin, especially on the neck, scalp, mucous membranes, underarms, and/or groin area. (For more information on this disorder, choose "Pemphigus" as your search term in the Rare Disease Database.)

Erythema multiforme is an allergic inflammatory skin disorder characterized by lesions that develop on the skin and/or mucous membranes. The early symptoms may include red, elevated spots (erythematous macules or papules) on the skin that may have fluid-filled centers. These papules eventually grow into larger blisters. Affected areas generally include the hands, forearms, feet, and/or mucous membranes of the mouth, nose, and/or genitals. The skin lesions and blisters caused by Erythema multiforme generally appear on both sides of the body. They tend to heal in approximately 2 to 6 weeks. Erythema multiforme may also cause fever, joint pain, cough, and a sore throat. (For more information on this disorder, choose "Erythema Multiforme" as your search term in the Rare Disease Database.)

Epidermolytic hyperkeratosis (bullous type) is a rare inherited skin disorder characterized by the overgrowth of skin (hyperkeratosis) and abnormal redness of the skin (erythroderma). The symptoms of this disorder are present at birth and may range from mild to severe. The skin may have warts or blisters and be abnormally thick, particularly in the skin creases over joints. (For more information on this disorder, choose "Epidermolytic Hyperkeratosis" as your search term in the Rare Disease Database.)

Epidermolysis bullosa refers to a group of rare skin diseases characterized by fragile skin, blistering, and small fluid-filled lesions that develop following minor trauma to the skin. The mucous membranes are also involved in some forms of Epidermolysis bullosa. Healing may be slow and blisters may leave multiple scars and/or damage the underlying muscle tissue. Most types of Epidermolysis bullosa are inherited and tend to develop during childhood. (For more information on these disorders, choose "Epidermolysis Bullosa" as your search term in the Rare Disease Database.)

Epidermolysis bullosa acquisita is a rare autoimmune skin disorder that typically affects middle-aged and elderly people. Trauma to the skin can cause blisters on the elbows, knees, pelvis, buttocks, and/or scalp. Increased levels of IgG (an immunoglobulin) are usually found around the blisters. After the blisters heal, scars usually remain. (For more information on this disorder, choose "Epidermolysis bullosa Acquisita" as your search term in the Rare Disease Database.)

The following disorder may precede the development of dermatitis herpetiformis. It can be useful in identifying an underlying cause of some forms of this disorder:

Celiac sprue is an inherited intestinal malabsorption disorder associated with intolerance to gluten. The symptoms may range from mild to severe and the age of onset from infancy to adulthood. Symptoms in children may include diarrhea, projectile vomiting, a bloated abdomen, and/or growth delays. Frequent symptoms in adults may include weight loss, diarrhea, excessively fatty stools, and abdominal cramping. Food cravings, weakness, and fatigue are also common. In some cases, lactose intolerance and/or dermatitis herpetiformis may develop in affected individuals. (For more information on this disorder, choose "Celiac Sprue" as your search term in the Rare Disease Database.)

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

Organizations related to Dermatitis Herpetiformis

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

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2008/01/29 00:00:00 GMT+0

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