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Dermatitis herpetiformis in Adult
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Dermatitis herpetiformis in Adult

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Contributors: Lauren Strazzula MD, Susan Burgin MD, Belinda Tan MD, PhD, Lowell A. Goldsmith MD, MPH
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Synopsis

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. The pathogenesis involves the deposition of IgA immune complexes in the papillary dermis. The associated autoantigen is an epidermal transglutaminase. There is a genetic predisposition to the disease, as certain human leukocyte antigen (HLA) haplotypes, HLA class II DQ2, demonstrate increased expression.

The disease manifests as an intermittent pruritic papulovesicular eruption over the extensor surfaces of the extremities. 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 Hashimoto thyroiditis, insulin-dependent diabetes mellitus, and enteropathy-associated T-cell lymphoma, and therefore warrant close surveillance.

A population-based study from Finland strongly suggests an increased risk of bullous pemphigoid in patients with dermatitis herpetiformis.

For more information, see OMIM.

Codes

ICD10CM:
L13.0 – Dermatitis herpetiformis

SNOMEDCT:
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 exam.
  • Pemphigoid gestationis is characterized by tense bullae over the abdomen in pregnant women.
  • Chronic prurigo 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.

Best Tests

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Management Pearls

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Therapy

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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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References

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Last Updated: 03/27/2019
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Dermatitis herpetiformis in Adult
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Dermatitis herpetiformis : Buttocks, Elbow, Extensor distribution, Knee, Skin erosion, Vesicle, Pruritus
Clinical image of Dermatitis herpetiformis
A close-up of excoriated papules / vesicles near the knee.
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