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Author: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, 2001. Updated by Dr Shendy Engelina, Core Medical Trainee, Northampton General Hospital, United Kingdom, February 2016.
Dermatitis herpetiformis is a rare but persistent immunobullous disease that has been linked to coeliac disease (the American spelling is celiac), a gluten-sensitive enteropathy.
The name herpetiformis is derived from the tendency for blisters to appear in clusters, resembling herpes simplex. However, dermatitis herpetiformis is not due to viral infection.
Dermatitis herpetiformis is also known as Duhring-Brocq disease.
The following conditions may affect patients with dermatitis herpetiformis, especially when it is associated with coeliac disease:
*The risk of NHL is increased among dermatitis herpetiformis patients. Strict adherence to gluten-free diet reduces this rare but serious long-term complication.
Skin biopsy is usually necessary to confirm dermatitis herpetiformis. Characteristic histological appearances of dermatitis herpetiformis are:
Patients with dermatitis herpetiformis are commonly offered the same blood tests used for patients with coeliac disease to screen for nutritional deficiencies. These include:
Mild anaemia may be caused by iron or folic acid deficiency (or both) due to malabsorption associated with gluten-sensitive enteropathy. Thyroid function tests are usually recommended due to the association between dermatitis herpetiformis and thyroid disease.
Specific autoantibody tests for dermatitis herpetiformis are:
*Dermatitis herpetiformis is associated with IgA antibodies directed against epidermal transglutaminase (eTG), which is not the case in coeliac disease.
Borderline results may be difficult to interpret.
HLA haplotype, a set of DNA variations, testing may reveal HLA-DQ2 or HLA-DQ8. This is present in almost all patients with dermatitis herpetiformis (and coeliac disease).
Gluten-free diet for life is strongly recommended in patients with dermatitis herpetiformis, as it:
Dapsone is the treatment of choice for dermatitis herpetiformis, as it usually reduces itch within 3 days.
If intolerant or allergic to dapsone, the following may be useful:
Dermatitis herpetiformis usually has a good prognosis, with the majority of patients responding well to a strict gluten-free diet and medication. The response rate varies between individuals (days to years).
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