Diagnosis
Dermatitis herpetiformis
A chronic autoimmune blistering disorder associated with a gluten-sensitive enteropathy (celiac disease).
Etiology
Cause [vdo]
Genetics
Environmental factors
Pathophysiology
IgA autoantibodies against transglutaminases deposited in the dermis
Immunologic cascade
Neutrophil recruitment and complement activation --> blistering disease
Associated with
Celiac disease, gastric atrophy, gastric hypochlorhydria, pernicious anemia, dermatomyositis, diabetes mellitus, myasthenia gravis, rheumatoid arthritis, Sjögren syndrome, systemic lupus erythematosus, hypothyroidism, hyperthyroidism, thyroid nodules, thyroid cancer and lymphoma
Epidemiology
Incidence per 100.000 [ftn][tcm][rsq][ktb][ii8][fbs][18n][cbc][qnb]
Symptoms & findings
Symptoms
Bloating, Diarrhea, Steatorrhea
Clinical findings
None listed.
Anamneses
None listed.
Localized findings
Approach
Biopsy with immunofluorescence (deposition of IgA)
Gastroscopy
Thyroid status
Glucose
Treatment
First-Line [vdo]
Gluten-free diet
Dapsone 25 to 150 mg daily
Potent topical steroids
Second-line
Sulphamethoxypyridazine 0.5 to 1.5 g daily (with a gluten-free diet)
Third-line
Sulphapyridine 250 to 750 mg daily or
Sulphasalazine 1 to 2 g daily (with a gluten-free diet)
Differential diagnoses
Bullous pemphigoid, Eczema, Epidermolysis bullosa, Erythema multiforme, Henoch-Schönlein purpura, IgA Pemphigus, Linear IgA dermatosis, Scabies, Transient acantholytic dermatosis, Urticaria
References
[1] Mirza HA, Gharbi A, Bhutta BS. Dermatitis Herpetiformis. [Updated 2023 Aug 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK493163/
[2] Albadri Z, Al Bayati D, Häbel H, Jerkovic Gulin S, Grönhagen C, Seifert O. Incidence of Dermatitis Herpetiformis in Sweden 2005 to 2018: A Nationwide Retrospective Cohort Study. Acta Derm Venereol. 2023 Nov 16;103:adv13210.
[3] Salmi TT, Hervonen K, Kautiainen H, Collin P, Reunala T. Prevalence and incidence of dermatitis herpetiformis: a 40-year prospective study from Finland. Br J Dermatol. 2011 Aug;165(2):354-9.
[4] West J, Fleming KM, Tata LJ, Card TR, Crooks CJ. Incidence and prevalence of celiac disease and dermatitis herpetiformis in the UK over two decades: population-based study. Am J Gastroenterol. 2014 May;109(5):757-68.
[5] Smith JB, Tulloch JE, Meyer LJ, Zone JJ. The incidence and prevalence of dermatitis herpetiformis in Utah. Arch Dermatol. 1992 Dec;128(12):1608-10.
[6] Mobacken H, Kastrup W, Nilsson LA. Incidence and prevalence of dermatitis herpetiformis in western Sweden. Acta Derm Venereol. 1984;64(5):400-4.
[7] Reunala T, Lokki J. Dermatitis herpetiformis in Finland. Acta Derm Venereol. 1978;58(6):505-10.
[8] Moi H. Incidence and prevalence of dermatitis herpetiformis in a country in central Sweden, with comments on the course of the disease and IgA deposits as diagnostic criterion. Acta Derm Venereol. 1984;64(2):144-50.
[9] Gawkrodger DJ, Blackwell JN, Gilmour HM, Rifkind EA, Heading RC, Barnetson RS. Dermatitis herpetiformis: diagnosis, diet and demography. Gut. 1984 Feb;25(2):151-7.
[10] Virta LJ, Saarinen MM, Kolho KL. Declining trend in the incidence of biopsy-verified coeliac disease in the adult population of Finland, 2005-2014. Aliment Pharmacol Ther. 2017 Dec;46(11-12):1085-1093.
[11] http://emedicine.medscape.com/article/1062640; [Medscape]
[12] [Clinical Dermatology 4.th Edition by Weller, Hunter, Savin and Dahl]