What are the other Names for this Condition? (Also known as/Synonyms)

• Brocq-Duhring Disease
• DH (Dermatitis Herpetiformis)
• Duhring's Disease

What is Dermatitis Herpetiformis? (Definition/Background Information)

• Dermatitis Herpetiformis (DH) is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. The lesions are more common on the knees, elbows, buttocks and shoulder blades
• Other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning
• The slow onset of symptoms usually begins during adulthood, but children can also be affected
• 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

(Source: Dermatitis Herpetiformis; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Dermatitis Herpetiformis? (Age and Sex Distribution)

• Dermatitis Herpetiformis is a rare disorder. The presentation of symptoms are typically seen in adults, but symptoms’ manifestation in childhood have also been reported
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Dermatitis Herpetiformis? (Predisposing Factors)

• Consumption of gluten-containing foods is a main risk factor for Dermatitis Herpetiformis
• At the present time, no other risk factors have been clearly identified for the disorder

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dermatitis Herpetiformis? (Etiology)

• The exact cause of Dermatitis Herpetiformis is not known, but the condition is frequently associated with the inability to digest gluten

(Source: Dermatitis Herpetiformis; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Dermatitis Herpetiformis?

The signs and symptoms of Dermatitis Herpetiformis vary, but may include:

Very frequently present symptoms in 80-99% of the cases:

• Abnormal blistering of the skin
• Autoimmunity
• Erythema
• Macule
• Malabsorption
• Microcytic anemia
• Pruritus
• Recurrent fractures
• Skin vesicle
• Urticaria

Frequently present symptoms in 30-79% of the cases: Eczema

Occasionally present symptoms in 5-29% of the cases:

• Bone pain
• Edema
• Lichenification

(Source: Dermatitis Herpetiformis; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Dermatitis Herpetiformis Diagnosed?

Dermatitis Herpetiformis is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dermatitis Herpetiformis?

The complications of Dermatitis Herpetiformis may include:

• Scarring and disfigurement
• Malnutrition
• An increased risk of osteoporosis
• Side effects of drugs used in the treatment of DH

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Dermatitis Herpetiformis Treated?

• The antibiotic dapsone is extremely effective in treating Dermatitis Herpetiformis, with possible symptomatic improvement in as little as several hours after the first dose. 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 
• In some cases, immunosuppressive medications may be used. These medications do not appear to be as effective 
• 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 

(Source: Dermatitis Herpetiformis; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Dermatitis Herpetiformis be Prevented?

• Currently, Dermatitis Herpetiformis may not be preventable, since the cause of this disorder is unknown.
• However, the onset of symptoms may be preventable by adhering to a gluten-free diet
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Dermatitis Herpetiformis? (Outcomes/Resolutions)

• The prognosis of Dermatitis Herpetiformis is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Dermatitis Herpetiformis:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/