Dermatitis herpetiformis Duhring

Last Updated: 2022-02-25

Author(s): Anzengruber F., Navarini A.

ICD11: EB44

  • Fox 1880
  • Duhring 1884

Dermatitis herpetiformis, Duhring's disease.

Autoimmune disease associated with gluten-sensitive enteropathy (coeliac disease) in which epidermal transglutaminase is the target antigen.

  • Prevalence (Northern Europe): 1.2 - 75.3/100000/year
  • Incidence (Northern Europe): 0.4 - 3.5/100000/year
  • Men : women = 1.1 - 1.9 : 1
  • Peak in incidence: 40-50 yrs, but all ages may be affected

  • IgA antibodies directed against epidermal transglutaminases are likely to play a role
  • Associations exists to:
    • Glut-sensitive enteropathy
    • HLA molecules-B8, -DQw2, -DR3 or, less commonly, to the DQ8 haplotype
    • Autoimmune thyroid diseases
    • Diabetes mellitus type I
    • Collagenoses
      • Lupus erythematosus
      • Sjörgren's syndrome
      • Vitiligo
  • Predisposing factors
    • Sensitivity to iodine
    • Foci
    • Malignant tumours (especially lymphomas)
    • Changes in the jejunum villi (intestinal changes are asymptomatic in dermatitis herpetiformis Duhring, at least in the initial stage)
  • Trigger factor
    • Gluten

  • Symmetrical, sometimes very pruritic and scratch-excoriated, often grouped papules, papulovesicles and plaques. The episodes can last from months to years. The oral mucosa is excluded
  • Symptoms of enteropathy are absent or only very mild (e.g. steatorrhoea)

  • Anamnesis
    • Associated diseases?
    • Improvement with gluten-free diet?
  • Blood count (optional: eosinophilia)
  • Clinic
  • Biopsy
    • Dermatopathology
    • Direct immunofluorescence
  • Serology
    • Antibody detection
      • Anti-gliadin-antibodies
      • Anti-endomysium-antibodies
      • Antibodies against tissue transglutaminase
      • Antibodies against epidermal transglutaminase (most sensitive blood test)

Capillitium, extensor sides of extremities, shoulders, in the lumbar spine region and gluteal.

  2. Eberhartinger C. Pemphigus vulgaris vom Typ der Dermatitis herpetiformis Duhring. Archiv für Klinische und Experimentelle Dermatologie 1957;206:778-.
  3. Rose C, Bröcker E-B, Zillikens D. Klinik, Histologie und Immunpathologie bei 32 Patienten mit Dermatitis herpetiformis Duhring. JDDG: Journal der Deutschen Dermatologischen Gesellschaft 2010;8:265-71.
  4. Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. Print.