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.

Gluten-free diet:

  • Advantages:
    • Reduction in dosage of systemic therapy or complete discontinuation possible after 2-3 years
    • Reduction in risk of developing lymphoma
    • If GI symptoms improve

Topical therapy:

  • Topical therapy alone is not promising
  • Remoisturing
  • Mometasone fuorate cream / solution / ointment
  • Clobetasol propionate cream 1x daily (for 1-3 days)

Systemic therapy:

  • No systemic glucocorticoids due to lack of efficacy!
  • Dapsone p.o. 1.5mg/ kg bw daily, if necessary increase every 2 weeks by 25-50mg, up to max. 300 mg daily
    • No preparations are currently approved in Switzerland
    • Before administration: exclusion of glucose-6-phosphate dehydrogenase deficiency
    • Met-Hb: should be checked 2 weeks and 6 weeks after starting therapy
  • Antihistamines
    • Levocetirizine p.o. 5 mg 1x daily
    • Desloratadine p.o. 5 mg 1x daily
    • Fexofenadine p.o. 180 mg 1x daily
  • Sulfasalazine p.o. 0.5 g
    • Initially: 1-2g 3-4x daily
    • Maintenance therapy: 1-2g 2-3x daily
  • Colchicine (not commercially available in Switzerland) p.o. 0.5 mg 3x daily
  • Ciclosporin p.o. 3-5 mg/kg bw daily
    • Take independently of meals
  • For gluten-induced enteropathy: internal medicine presentation

  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.