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Dermatitis herpetiformis is an uncommon disease manifested by intensely pruritic papules, vesicles, and papulovesicles mainly on the elbows, knees, buttocks, posterior neck, and scalp (eFigure 6–89). It has the highest prevalence in Northern Europe and is associated with HLA antigens -B8, -DR3, and -DQ2. The histopathology is distinctive. Circulating antibodies to tissue transglutaminase are present in 90% of cases. NSAIDs may cause flares. Patients may have gluten-sensitive enteropathy. Three-fourths of patients have villous atrophy on small bowel biopsy; however, GI symptoms are subclinical in most. The prevalence of dermatitis herpetiformis to celiac disease is 1:8. Ingestion of gluten is the cause of dermatitis herpetiformis, and strict long-term avoidance of dietary gluten may eliminate the need for treatment or decrease the dose of dapsone (initial treatment dose is 100–200 mg orally daily) required to control the disease. Patients with dermatitis herpetiformis are at increased risk for GI lymphoma, and this risk is reduced by a gluten-free diet.

eFigure 6–89.

Dermatitis herpetiformis presenting typically as pruritic papulovesicles on this Scandinavian man's elbow. (Used, with permission, from J Reeves, MD.)

Reunala  T  et al. Dermatitis herpetiformis: an update on diagnosis and management. Am J Clin Dermatol. 2021;22:329.
[PubMed: 33432477]  
Salmi  T  et al. Current concepts of dermatitis herpetiformis. Acta Derm Venereol. 2020;100:adv00056.
[PubMed: 32039457]  

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