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Dermatitis Herpetiformis

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Autoimmune Bullous Diseases

Abstract

Dermatitis herpetiformis is an immunobullous skin disease that is closely related to gluten sensitive enteropathy. Classic clinical characteristics of intensely pruritic vesiculopapules on the elbows, knees, and scalp are well defined, although some clinical variation exists. In most cases the diagnosis is firmly established by the presence of granular IgA in the dermal papillae on direct immunofluorescent studies. Sulfonamide compounds were first described as effective therapy several decades ago and remain the mainstay of medical therapy, while a strict gluten free diet will keep patients free of cutaneous manifestations in the majority of cases. Our understanding of dermatitis herpetiformis continues to grow, as does our understanding of the relationship between the gut and the skin.

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Abbreviations

DH:

Dermatitis herpetiformis

DIF:

Direct immunofluorescence

GSE:

Gluten sensitive enteropathy

H&E:

Hematoxylin and eosin

IgA:

Immunoglobulin A

References

  1. Duhring LA. Dermatitis herpetiformis. JAMA. 1884;3(9):212–6.

    Google Scholar 

  2. Kalis JB, Malkinson FD. Landmark perspective: dermatitis herpetiformis. A commentary. JAMA [United States]. 1983;250(2):217–21.

    Article  CAS  Google Scholar 

  3. COSTELLO MJ. Dermatitis herpetiformis (papular type) successfully treated with sulfapyridine. Arch Derm Syphilol. 1947;55(5):725. Not Available.

    CAS  PubMed  Google Scholar 

  4. Marks J, Shuster S, Watson AJ. Small-bowel changes in dermatitis herpetiformis. Lancet [England]. 1966;2(7476):1280–2.

    Article  CAS  Google Scholar 

  5. Fry L, McMinn RM, Cowan JD, Hoffbrand AV. Effect of gluten-free diet on dermatological, intestinal, and haematological manifestations of dermatitis herpetiformis. Lancet [England]. 1968;1(7542):557–61.

    Article  CAS  Google Scholar 

  6. Reunala T, Lokki J. Dermatitis herpetiformis in Finland. Acta Derm Venereol [Sweden]. 1978;58(6):505–10.

    CAS  Google Scholar 

  7. Mobacken H, Kastrup W, Nilsson LA. Incidence and prevalence of dermatitis herpetiformis in western Sweden. Acta Derm Venereol [Sweden]. 1984;64(5):400–4.

    CAS  Google Scholar 

  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 [Sweden]. 1984;64(2):144–50.

    CAS  Google Scholar 

  9. 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 [United States]. 2014;109(5):757–68.

    Article  Google Scholar 

  10. 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 [Internet]. 2011;165(2):354–9. [cited 2014 Dec 14].

    Article  CAS  Google Scholar 

  11. Hall RP, Clark RE, Ward FE. Dermatitis herpetiformis in two American blacks: HLA type and clinical characteristics. J Am Acad Dermatol [United States]. 1990;22(3):436–9.

    Article  CAS  Google Scholar 

  12. Ohata C, Ishii N, Hamada T, Shimomura Y, Niizeki H, Dainichi T, et al. Distinct characteristics in Japanese dermatitis herpetiformis: a review of all 91 Japanese patients over the last 35 years. Clin Dev Immunol [Internet]. 2012;2012:562168. [cited 2014 Dec 14].

    Google Scholar 

  13. Gawkrodger DJ, Blackwell JN, Gilmour HM, Rifkind EA, Heading RC, Barnetson RS. Dermatitis herpetiformis: diagnosis, diet and demography. Gut [England]. 1984;25(2):151–7.

    Article  CAS  Google Scholar 

  14. Salmi TT, Hervonen K, Laurila K, Collin P, Mäki M, Koskinen O, et al. Small bowel transglutaminase 2-specific IgA deposits in dermatitis herpetiformis. Acta Derm Venereol [Internet]. 2014;94(4):393–7. [cited 2014 Dec 14].

    Article  Google Scholar 

  15. Katz SI, Falchuk ZM, Dahl MV, Rogentine GN, Strober W, Service D, et al. HL-A8: a genetic link between dermatitis herpetiformis and gluten-sensitive enteropathy. J Clin Invest. 1972;51(11):2977–80.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Spurkland A, Ingvarsson G, Falk ES, Knutsen I, Sollid LM, Thorsby E. Dermatitis herpetiformis and celiac disease are both primarily associated with the HLA-DQ (alpha 1*0501, beta 1*02) or the HLA-DQ (alpha 1*03, beta 1*0302) heterodimers. Tissue Antigens [Denmark]. 1997;49(1):29–34.

    Article  CAS  Google Scholar 

  17. Reunala T, Kosnai I, Karpati S, Kuitunen P, Torok E, Savilahti E. Dermatitis herpetiformis: jejunal findings and skin response to gluten free diet. Arch Dis Child [Internet]. 1984;59(6):517–22.

    Article  CAS  Google Scholar 

  18. Alonso-llamazares J, Gibson LE, Rogers III RS. Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience. Int J Dermatol. 2007;46:910–9.

    Article  PubMed  Google Scholar 

  19. Garioch JJ, Lewis HM, Sargent SA, Leonard JN, Fry L. 25 years’ experience of a gluten-free diet in the treatment of dermatitis herpetiformis. Br J Dermatol [England]. 1994;131(4):541–5.

    Article  CAS  Google Scholar 

  20. Lorand L, Graham RM. Transglutaminases: crosslinking enzymes with pleiotropic functions. Nat Rev Mol Cell Biol [Internet]. 2003;4(2):140–56. [cited 2014 Dec 8]. Available from

    Google Scholar 

  21. Martin A, De Vivo G, Gentile V. Possible role of the transglutaminases in the pathogenesis of Alzheimer’s disease and other neurodegenerative diseases. Int J Alzheimers Dis [Internet]. 2011;2011(Cd):865432. [cited 2014 Dec 7].

    Google Scholar 

  22. Alaedini A, Green PHR. Narrative review: celiac disease: understanding a complex autoimmune disorder. Ann Intern Med [United States]. 2005;142(4):289–98.

    Article  CAS  Google Scholar 

  23. Porter WM, Unsworth DJ, Lock RJ, Hardman CM, Baker BS, Fry L. Tissue transglutaminase antibodies in dermatitis herpetiformis Gastroenterology. 1999;117(3):749–50.

    Google Scholar 

  24. Sardy M, Karpati S, Merkl B, Paulsson M, Smyth N. Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis. J Exp Med [United States]. 2002;195(6):747–57.

    Article  CAS  Google Scholar 

  25. Rose C, Armbruster FP, Ruppert J, Igl BW, Zillikens D, Shimanovich I. Autoantibodies against epidermal transglutaminase are a sensitive diagnostic marker in patients with dermatitis herpetiformis on a normal or gluten-free diet. J Am Acad Dermatol [Internet]. 2009;61(1):39–43. Elsevier Inc. [cited 2014 Dec 14].

    Article  CAS  Google Scholar 

  26. Zone JJ, Egan CA, Taylor TB, Meyer LJ. Iga autoimmune disorders: development of a passive transfer mouse model. J Investig Dermatology Symp Proc. 2004;9(1):47–51.

    Article  CAS  Google Scholar 

  27. Heinlin J, Knoppke B, Kohl E, Landthaler M, Karrer S. Dermatitis herpetiformis presenting as digital petechiae. Pediatr Dermatol [Internet]. 2012;29(2):209–12. [cited 2014 Dec 14].

    Article  Google Scholar 

  28. Flann S, Degiovanni C, Derrick EK, Munn SE. Two cases of palmar petechiae as a presentation of dermatitis herpetiformis. Clin Exp Dermatol [Internet]. 2010;35(2):206–8. [cited 2014 Dec 14].

    Article  CAS  Google Scholar 

  29. Blenkinsopp WK, Haffenden GP, Fry L, Leonard JN. Histology of linear IgA disease, dermatitis herpetiformis, and bullous pemphigoid. Am J Dermatopathol [United States]. 1983;5(6):547–54.

    Article  CAS  Google Scholar 

  30. Warren SJP, Cockerell CJ. Characterization of a subgroup of patients with dermatitis herpetiformis with nonclassical histologic features. Am J Dermatopathol. 2002;24(4):305–8.

    Article  PubMed  Google Scholar 

  31. Huber C, Trueb RM, French LE, Hafner J. Negative direct immunofluorescence and nonspecific histology do not exclude the diagnosis of dermatitis herpetiformis Duhring. Int J Dermatol [England]. 2013;52:248–9.

    Article  Google Scholar 

  32. Katz SI, Hertz KC, Crawford PS, Gazze LA, Frank MM, Lawley TJ. Effect of sulfones on complement deposition in dermatitis herpetiformis and on complement-mediated guinea-pig reactions. J Invest Dermatol [United States]. 1976;67(6):688–90.

    Article  CAS  Google Scholar 

  33. Mery L, Dega H, Prost C, Dubertret L. [Dapsone-induced sensory peripheral neuropathy]. Ann Dermatol Venereol [France]. 2003;130(4):447–9.

    CAS  Google Scholar 

  34. Willsteed E, Lee M, Wong LC, Cooper A. Sulfasalazine and dermatitis herpetiformis. Australas J Dermatol [Internet]. 2005;46(2):101–3.

    Article  Google Scholar 

  35. Silvers DN, Juhlin EA, Berczeller PH, McSorley J. Treatment of dermatitis herpetiformis with colchicine. Arch Dermatol [United States]. 1980;116(12):1373–84.

    Article  CAS  Google Scholar 

  36. Zemtsov A, Neldner KH. Successful treatment of dermatitis herpetiformis with tetracycline and nicotinamide in a patient unable to tolerate dapsone. J Am Acad Dermatol [United States]. 1993;28(3):505–6.

    Article  CAS  Google Scholar 

  37. Fry L, Seah PP, Riches DJ, Hoffbrand AV. Clearance of skin lesions in dermatitis herpetiformis after gluten withdrawal. Lancet [England]. 1973;1(7798):288–91.

    Article  CAS  Google Scholar 

  38. Kastrup W, Mobacken H, Stockbrugger R, Swolin B, Westin J. Malabsorption of vitamin B12 in dermatitis herpetiformis and its association with pernicious anaemia. Acta Med Scand [Sweden]. 1986;220(3):261–8.

    Article  CAS  Google Scholar 

  39. Denman SL, Freeman NJ. Asymptomatic anemia in three men with dermatitis herpetiformis. Hosp Pract [United States]. 1992;27(4):187, 189–90.

    CAS  Google Scholar 

  40. Krishnareddy S, Lewis SK, Green PH. Dermatitis herpetiformis: clinical presentations are independent of manifestations of celiac disease. Am J Clin Dermatol [Internet]. 2014;15(1):51–6. [cited 2014 Dec 14].

    Article  Google Scholar 

  41. Gawkrodger DJ, Ferguson A, Barnetson RS. Nutritional status in patients with dermatitis herpetiformis. Am J Clin Nutr [United States]. 1988;48(2):355–60.

    CAS  Google Scholar 

  42. Neuhausen SL, Steele L, Ryan S, Mousavi M, Pinto M, Osann KE, et al. Co-occurrence of celiac disease and other autoimmune diseases in celiacs and their first-degree relatives. J Autoimmun [England]. 2008;31(2):160–5.

    Article  CAS  Google Scholar 

  43. Reunala T, Collin P. Diseases associated with dermatitis herpetiformis. Br J Dermatol [England]. 1997;136(3):315–8.

    Article  CAS  Google Scholar 

  44. Leonard JN, Tucker WF, Fry JS, Coulter CA, Boylston AW, McMinn RM, et al. Increased incidence of malignancy in dermatitis herpetiformis. Br Med J (Clin Res Ed) [England]. 1983;286(6358):16–8.

    Article  CAS  Google Scholar 

  45. Sigurgeirsson B, Agnarsson BA, Lindelof B. Risk of lymphoma in patients with dermatitis herpetiformis. BMJ Clin Res. 1994;308:13–5.

    Article  CAS  Google Scholar 

  46. Lewis HM, Renaula TL, Garioch JJ, Leonard JN, Fry JS, Collin P, et al. Protective effect of gluten-free diet against development of lymphoma in dermatitis herpetiformis. Br J Dermatol [England]. 1996;135(3):363–7.

    Article  CAS  Google Scholar 

  47. Bardella MT, Fredella C, Trovato C, Ermacora E, Cavalli R, Saladino V, et al. Long-term remission in patients with dermatitis herpetiformis on a normal diet. Br J Dermatol [England]. 2003;149(5):968–71.

    Article  CAS  Google Scholar 

  48. Paek SY, Steinberg SM, Katz SI. Remission in dermatitis herpetiformis: a cohort study. Arch Dermatol [Internet]. 2011;147(3):301–5. [cited 2014 Dec 14].

    Article  Google Scholar 

  49. McAllister CS, Kagnoff MF. The immunopathogenesis of celiac disease reveals possible therapies beyond the gluten-free diet. Semin Immunopathol [Internet]. 2012;34(4):581–600. [cited 2014 Dec 14].

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Dermatology, University of Pittsburgh, 580 South Aiken Ave., Suite 200, Pittsburgh, PA, 15232, USA

    Timothy Patton DO

  2. Department of Dermatology, University Hospitals Case Medical Center, Cleveland, OH, USA

    Neil J. Korman MD, PhD

Authors
  1. Timothy Patton DO
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  2. Neil J. Korman MD, PhD
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Correspondence to Timothy Patton DO .

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Editors and Affiliations

  1. Department of Dermatology, University of Alabama at Birmingham, BIRMINGHAM, Alabama, USA

    Naveed Sami

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Patton, T., Korman, N.J. (2016). Dermatitis Herpetiformis. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_10

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  • DOI: https://doi.org/10.1007/978-3-319-26728-9_10

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