Dermatitis herpetiformis Duhring
Last Updated: 2022-02-25
- 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
- Lupus erythematosus
- Sjörgren's syndrome
- Predisposing factors
- Sensitivity to iodine
- 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
- 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)
- Associated diseases?
- Improvement with gluten-free diet?
- Blood count (optional: eosinophilia)
- Direct immunofluorescence
- Antibody detection
- Antibodies against tissue transglutaminase
- Antibodies against epidermal transglutaminase (most sensitive blood test)
- Antibody detection
Capillitium, extensor sides of extremities, shoulders, in the lumbar spine region and gluteal.
- 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 alone is not promising
- Mometasone fuorate cream / solution / ointment
- Clobetasol propionate cream 1x daily (for 1-3 days)
- 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
- 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
- Duhring LA. DERMATITIS HERPETIFORMIS. JAMA 1884;III:225.
- Eberhartinger C. Pemphigus vulgaris vom Typ der Dermatitis herpetiformis Duhring. Archiv für Klinische und Experimentelle Dermatologie 1957;206:778-.
- 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.
- Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. Print.