Dermatitis herpetiformis

Author: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Updated by Dr Shendy Engelina, Core Medical Trainee, Northampton General Hospital, UK, February 2016.

What is dermatitis herpetiformis?

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.

Who gets dermatitis herpetiformis?

What causes dermatitis herpetiformis?

What are the clinical features of dermatitis herpetiformis?

What are the complications of DH and coeliac 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.

How is dermatitis herpetiformis diagnosed?

Skin biopsy

Skin biopsy is usually necessary to confirm DH. Characteristic histological appearances of DH are:

Screen for nutritional deficiencies

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.

Diagnostic blood tests

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.

Small intestinal biopsy

Other tests

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).

What is the treatment for dermatitis herpetiformis?

Gluten-free diet

Gluten-free diet for life is strongly recommended in patients with DH, as it:

Medication

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:

What is the outcome for dermatitis herpetiformis?

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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