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Dermatitis Herpetiformis: A Common Extraintestinal Manifestation of Coeliac Disease

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Review

Dermatitis Herpetiformis: A Common Extraintestinal Manifestation of Coeliac Disease

Timo Reunala et al. Nutrients.

Abstract

Dermatitis herpetiformis (DH) is a common extraintestinal manifestation of coeliac disease presenting with itchy papules and vesicles on the elbows, knees, and buttocks. Overt gastrointestinal symptoms are rare. Diagnosis of DH is easily confirmed by immunofluorescence biopsy showing pathognomonic granular immunoglobulin A (IgA) deposits in the papillary dermis. A valid hypothesis for the immunopathogenesis of DH is that it starts from latent or manifest coeliac disease in the gut and evolves into an immune complex deposition of high avidity IgA epidermal transglutaminase (TG3) antibodies, together with the TG3 enzyme, in the papillary dermis. The mean age at DH diagnosis has increased significantly in recent decades and presently is 40⁻50 years. The DH to coeliac disease prevalence ratio is 1:8 in Finland and the United Kingdom (U.K.). The annual DH incidence rate, currently 2.7 per 100,000 in Finland and 0.8 per 100,000 in the U.K., is decreasing, whereas the reverse is true for coeliac disease. The long-term prognosis of DH patients on a gluten-free diet is excellent, with the mortality rate being even lower than for the general population.

Keywords: coeliac disease; dermatitis herpetiformis; epidermal transglutaminase; gluten-free diet; long-term prognosis; prevalence.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Dermatitis herpetiformis. Typical scratched papules and macules on the elbows (A), and on the knees (B). Fresh small blisters on the elbow (C). Direct immunofluorescence showing granular IgA deposits in the basal membrane zone between epidermis and dermis (D).
Figure 2
Figure 2
Incidence of dermatitis herpetiformis and coeliac disease in Finland, 1980–2014. The data include 3671 adult patients with Dermatitis herpetiformis and 31,385 adult patients with coeliac disease registered with the Social Insurance Institution of Finland [28,43].

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