Core Dermatology Diagnoses / CategoriesInflammatory

Dermatitis herpetiformis

Author: Faculty Reviewer: Resident Reviewer:

Publish date: Posted on
Last updated: May 31, 2022

Keywords #

dermatitis herpetiformis
celiac disease
autoimmune
blistering
gluten insensitivity

Diagnosis #

Dermatitis herpetiformis is an uncommon autoimmune blistering disease characterized by pruritic papules and vesicles mostly on the elbows, knees, and buttocks. A majority of patients also have an associated gluten-sensitive enteropathy (celiac disease) but most are minimally symptomatic or asymptomatic. The etiology of this disease is considered complex, involving both intrinsic and extrinsic factors. Certain HLA genes predispose a patient to developing dermatitis herpetiformis, with nearly all patients carrying the HLA-DQ2 or HLA-DQ8 haplotype.[1]

Key Concepts #
  • Dermatitis herpetiformis is an autoimmune blistering disease.
  • It is associated with celiac disease, a gluten-sensitive enteropathy.
  • It manifests as grouped pruritic papules and vesicles.
  • Diagnosis is based on clinical evaluation and laboratory studies, including direct immunofluorescence from a perilesional biopsy specimen.
  • The mainstay of treatment includes dapsone therapy and a gluten-free diet.
Epidemiology #

DH is an uncommon skin disorder that most frequently affects individuals of northern European heritage. Prevalence rates in northern Europe were found to be 75.3 per 100,000 people per year.[2] Incidence rates were found to range from 0.8 to 2.7 per 100,000 people per year in the United Kingdom and Finland.[2-3] It is more common in males than females, and less likely to be diagnosed during childhood.

Clinical Features #
  • DH classically manifests as multiple grouped intensely pruritic papules and vesicles, most commonly occurring on the elbows, dorsal forearms, knees, scalp, back, and buttocks.[4]
  • Erosions and excoriations are seen on physical exam due to the associated pruritus.
  • Mild disease may present with few localized areas, whereas severe disease may present with widely distributed lesions on the trunk or extremities.
  • These lesions typically heal without scarring but may develop post inflammatory hyperpigmentation or hypopigmentation.
Differential Diagnoses #
Diagnostic Workup #

Diagnosis is based on clinical findings and laboratory studies, including tissue pathology, direct immunofluorescence microscopy, and serology. While the rash is typically vesicular, due to scratching, the vesicles are often broken and show only erosions and crusts. Diagnosis should be confirmed with perilesional skin biopsy and direct immunofluorescence examination, which classically shows granular IgA in the papillary dermis.[5] Serologic studies can also help confirm the diagnosis by showing elevated levels of IgA tissue glutaminase antibodies, IgA epidermal transglutaminase antibodies, and IgA endomysial antibodies.

Treatment #

The two mainstays of treatment include a strict gluten-free diet and dapsone therapy.[6-7] Dapsone is rapidly effective and results in resolution of active skin lesions, sometimes within days. Over time, dapsone can be tapered with the goal of maintaining clinical improvement with diet alone.

References #
  1. Bonciani D, Verdelli A, Bonciolini V, et al. Dermatitis Herpetiformis: From the Genetics to the Development of Skin Lesions. Clin Dev Immunol. 2012;2012:1-7. doi:10.1155/2012/239691.
  2. Salmi TT, Hervonen K, Kautiainen H et al. Prevalence and incidence of dermatitis herpetiformis: a 40‐year prospective study from Finland. Br J Dermatol 2011; 165: 354–9.
  3. West J, Fleming KM, Tata LJ et al. Incidence and prevalence of celiac disease and dermatitis herpetiformis in the UK over two decades: a population‐based study. Am J Gastroenterol 2014; 109: 757–68.
  4. Bolotin D, Petronic‐Rosic V. Dermatitis herpetiformis. Part I. Epidemiology, pathogenesis, and clinical presentation. J Am Acad Dermatol 2011; 64: 1017–24.
  5. Zone JJ, Meyer LJ, Petersen MJ. Deposition of granular IgA relative to clinical lesions in dermatitis herpetiformis. Arch Dermatol1996; 132: 912–18.
  6. Fry L, Leonard JN, Swain F, et al. Long term follow-up of dermatitis herpetiformis with and without dietary gluten withdrawal. Br J Dermatol. 1982;107(6):631-640. doi:10.1111/j.1365-2133.1982.tb00520.x.
  7. Zone JJ, Meyer LJ. Dermatitis herpetiformis. Immunol Ser. 1989;46:565-582. https://www.ncbi.nlm.nih.gov/pubmed/2488870. Accessed August 18, 2019.
Contents