Duhring Brocq disease


(Duhring Brocq disease is aka "Dermatitis herpetiformis", "Duhring's disease")

Dermatitis herpetiformis (DH) is a chronic disease of the skin marked by groups of watery, itchy blisters that may resemble pimples or blisters. The ingestion of gluten (from wheat, rye, and barley) triggers an immune system response that deposits a substance, lgA (Immunoglobulin A), under the top layer of skin. IgA is present in affected as well as unaffected skin. DH is a hereditary autoimmune gluten intolerance disease linked with celiac disease. If you have DH, you always have gluten intolerance. With DH, the primary lesion is on the skin, whereas with celiac disease the lesions are in the small intestine. The degree of damage to the small intestine is often less severe or more patchy than those with celiac disease. Both diseases are permanent and symptoms/damage will occur after consuming gluten.


Source: The Gluten Intolerance Group®


The lgA deposits under the skin result in eruptions of red raised patches of skin, similar to the beginning of a pimple, that can develop into small watery blisters. The itching and burning of the eruptions are severe and the urge to scratch them is intense. Scratching will further irritate the eruptions. Eruptions commonly occur on pressure points, such as around the elbows, the front of the knees, the buttocks, back face, and scalp but can appear anywhere on the body. Eruptions are usually bilateral - occurring on both sides of the body. 60% of those diagnosed are men and the most common age at diagnosis is 15 to 40 years old. Although it is uncommon to diagnose young children with DH, we are seeing more cases of early childhood DH.


Source: The Gluten Intolerance Group®


DH is caused by the deposit of IgA in the skin, which triggers further immunologic reactions resulting in lesion formation. DH is an external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin antigen epidermal transglutaminase.


Source: National Digestive Diseases Information Clearinghouse (NDDIC)


A skin biopsy is the first step in diagnosing DH. Direct immunofluorescence of clinically normal skin adjacent to a lesion shows granular IgA deposits in the upper dermis. Histology of lesional skin may show microabscesses containing neutrophils and eosinophils but may reveal only excoriation due to the intense itching patients experience.

Skin biopsies performed on the affected skin are nearly always positive for IgA deposition. Blood tests for antiendomysial or anti-tissue transglutaminase antibodies may also suggest celiac disease.

A positive biopsy and serology confirm celiac disease. In the absence of these results, patients should be referred to a gastroenterologist for a definitive diagnosis via intestinal biopsy.


Source: National Digestive Diseases Information Clearinghouse (NDDIC)


The disease may be well controlled with treatment. Without treatment, the risk of intestinal cancer may be significant.


Source: Medline Plus


Dapsone, a sulfone, provides immediate relief of symptoms. For patients who cannot tolerate dapsone, sulfapyridine or sulfamethoxypyridazine may be used, although these drugs are less effective than dapsone.

A strict gluten-free diet is the only treatment for the underlying disease. Even with a gluten-free diet, drug therapy may need to be continued for 1 to 2 years.


Source: National Digestive Diseases Information Clearinghouse (NDDIC)