Dermatitis herpetiformis

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.

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?

 DH predominantly affects Caucasians aged 15–40 years, but may occur in those younger or older and in
other races.
 There is a 2:1 male-to-female ratio.
 More females under the age of 20 are affected than males.
 There is a genetic predisposition and association with human leukocyte antigens (HLAs) DQ2 and DQ8.
 Some patients have a personal or family history of other autoimmune disorders including thyroid disease,
pernicious anaemia, type 1 diabetes, vitiligo, Addison disease and alopecia areata.
What causes dermatitis herpetiformis?
 DH and coeliac disease are due to intolerance to the gliadin fraction of gluten found in wheat, rye and
barley.
 Gluten triggers production of IgA antibodies and an autoimmune process that targets the skin and gut.
 In coeliac disease, gluten causes intestinal inflammation resulting in diarrhoea, tiredness, weight loss and
abdominal discomfort.
 The majority (> 90%) of patients with DH also have gluten-sensitive enteropathy. Gastrointestinal symptoms
may be mild to severe; some patients remain symptom-free.
 Around 15–25% coeliac patients have concurrent DH. These patients tend to have a more severe
intestinal pathology in comparison to those with mainly DH.

What are the clinical features of dermatitis herpetiformis?

 DH has a symmetrical distribution.
 Lesions most commonly appear on scalp, shoulders, buttocks, elbows and knees.
 It is characterised by prurigo (extremely itchy papules) and vesicles on normal or reddened skin.
 They often appear in groups or serpiginous clusters.
 Blisters are often eroded and crusted due to immediate scratching.
 DH may also present initially as digital petechiae.
 Flat red patches, thickened plaques and wheals may occur resembling other inflammatory skin conditions
such as dermatitis, scabies and papular urticaria.
 Lesions resolve to leave postinflammatory hypopigmentation and hyperpigmentation.