Dermatitis herpetiformis Duhring

Last Updated: 2022-02-25

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

ICD11: EB44

1/37

  • 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.

  1. Duhring LA. DERMATITIS HERPETIFORMIS. JAMA 1884;III:225.
  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.