Dermatitis herpetiformis Duhring

• 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

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.