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 (DH) is a rare but persistent immunobullous disease that has been linked to coeliac disease (American spelling celiac), a gluten-sensitive enteropathy.
The name herpetiformis is derived from the tendency for blisters to appear in clusters, resembling herpes simplex. However, DH is not due to viral infection.
DH is also known as Duhring-Brocq disease.
The following conditions may affect patients with DH, especially when it is associated with coeliac disease:
*The risk of NHL is increased among DH patients. Strict adherence to gluten-free diet reduces this rare but serious long-term complication.
Patients with DH 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 DH and thyroid disease.
Specific autoantibody tests for DH are:
*DH 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 DH (and coeliac disease).
Gluten-free diet for life is strongly recommended in patients with DH, as it:
Dapsone is the treatment of choice for DH, as it usually reduces itch within 3 days.
If intolerant or allergic to dapsone, the following may be useful:
DH usually has 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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