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Dermatitis herpetiformis in Adult
Other Resources UpToDate PubMed

Dermatitis herpetiformis in Adult

Contributors: Erin X. Wei MD, Lauren Strazzula MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed


Dermatitis herpetiformis (DH) is a chronic, pruritic autoimmune blistering disorder associated with gluten-sensitive enteropathy. DH most commonly affects patients of Northern European descent and occurs more frequently in men than women. While more than 90% of patients have associated gluten-sensitive enteropathy on small bowel biopsy, only 20% exhibit symptoms of intestinal disease at the time of diagnosis. The pathogenesis involves the deposition of IgA immune complexes in the papillary dermis. The associated autoantigen is an epidermal transglutaminase, transglutaminase 3 (TG3). There is a genetic predisposition to the disease, as it is associated with certain human leukocyte antigen (HLA) haplotypes, HLA class II DQ2.

The disease manifests as an intermittent pruritic, papulovesicular eruption over the extensor surfaces of the extremities, buttocks, and scalp. Many patients who adhere to a strict gluten-free diet experience complete remission of their disease.

Patients with DH are at an increased risk of developing thyroid disease, most commonly Hashimoto thyroiditis. Insulin-dependent diabetes mellitus and pernicious anemia have also been reported. Patients with gluten-sensitive enteropathy are also at increased risk of enteropathy-associated T-cell lymphoma and therefore warrant close surveillance. Gluten avoidance may decrease the risk for lymphoma. A population-based study from Finland strongly suggests an increased risk of bullous pemphigoid in patients with DH.


L13.0 – Dermatitis herpetiformis

111196000 – Dermatitis herpetiformis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Scabies manifests with interdigital burrows and involvement of the hands, wrists, and genital region, sparing the head.
  • Papular urticaria may be associated with arthropod bites and is distributed over exposed areas.
  • Bullous pemphigoid presents with urticarial erythematous plaques and intact, tense bullae in older patients.
  • Linear IgA bullous dermatosis presents with grouped vesicles and bullae, classically in an annular configuration.
  • Atopic dermatitis is also pruritic, with ill-defined, weeping, erythematous plaques.
  • Herpes simplex virus has nonsymmetric, localized, clustered vesicles with more pain and less pruritus.
  • Epidermolysis bullosa acquisita may have associated milia.
  • Transient acantholytic dermatosis (Grover disease) is a pruritic papular eruption in the seborrheic regions of older men.
  • Neurotic excoriations are distributed in areas within reach for the patient to scratch, and no primary lesions are appreciated on examination.
  • Pemphigoid gestationis is characterized by tense bullae over the abdomen in pregnant patients.
  • Chronic Prurigo nodularis consists of lichenified excoriated papules or nodules.
  • Prurigo pigmentosa usually affects the back, chest, and neck; the forehead and arms are rarely involved.
  • Evaluate for other causes of Pruritus without rash.

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed:02/05/2020
Last Updated:01/12/2022
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Dermatitis herpetiformis in Adult
A medical illustration showing key findings of Dermatitis herpetiformis : Buttocks, Elbow, Excoriation, Extensor distribution, Knee, Skin erosion, Vesicle, Pruritus
Clinical image of Dermatitis herpetiformis - imageId=117824. Click to open in gallery.  caption: 'A close-up of excoriated papules / vesicles near the knee.'
A close-up of excoriated papules / vesicles near the knee.
Copyright © 2024 VisualDx®. All rights reserved.