Abstract
Dermatitis herpetiformis (DH) is a chronic, polymorphic, blistering, and pruritic skin disease that develops mostly in patients with a latent gluten-sensitive enteropathy (GSE) called celiac disease. DH patients usually present with skin manifestations only and are not aware of the underlying gluten sensitivity. Perilesional granular IgA deposition at the uppermost papillary layer and lesional neutrophilic accumulation with subepidermal blister formation together prove the diagnosis. Almost all DH patients have circulating gluten-induced transglutaminase 2 (TG2) or endomysium antibodies characteristic for GSE, but they also have anti-TG3 IgA antibodies present only in some celiac patients. Associated autoimmunities, endocrinological, neurological, gastrointestinal pathologies need a careful multidisciplinary follow-up. Family screening for gluten sensitivity by IgA TG2 antibodies or exceptionally by the characteristic HLA background verification is suggested.
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References
Kárpáti S. Dermatitis herpetiformis: close to unraveling a disease. J Dermatol Sci. 2004;34:83–90.
Salmi TT, Hervonen K, Kautiainen H, et al. Prevalence and incidence of dermatitis herpetiformis: a 40-year prospective study from Finland. Br J Dermatol. 2011;165:354–9.
Ohata C, Ishii N, Hamada T, et al. Distinct characteristics in Japanese dermatitis herpetiformis: a review of all 91 Japanese patients over the last 35 years. Clin Dev Immunol. 2012;2012:562168. Published online 2012 June 12.
Junkins-Hopkins JM. Dermatitis herpetiformis: pearls and pitfalls in diagnosis and management. J Am Acad Dermatol. 2010;63:526–8.
Cardones AR, Hall 3rd RP. Management of dermatitis herpetiformis. Immunol Allergy Clin North Am. 2012;32:275–81.
Hervonen K, Alakoski A, Salmi TT, et al. Reduced mortality in dermatitis herpetiformis: a population-based study of 476 patients. Br J Dermatol. 2012;167:1331–7.
Paek SY, Steinberg SM, Katz SI. Remission in dermatitis herpetiformis: a cohort study. Arch Dermatol. 2011;147:301–5.
Mobacken H, Andersson H, Dahlberg E, et al. Spontaneous remission of dermatitis herpetiformis: dietary and gastrointestinal studies. Acta Derm Venereol. 1986;66:245–50.
Hervonen K, Vornanen M, Kautiainen H, et al. Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives. Br J Dermatol. 2005;152:82–6.
Zingone F, Bucci C, Tortora R, et al. Body mass index and prevalence of skin diseases in adults with untreated coeliac disease. Digestion. 2009;80:18–24.
Alakoski A, Salmi TT, Hervonen K. Chronic gastritis in dermatitis herpetiformis: a controlled study. Clin Dev Immunol. 2012;2012:640630. Epub 2012 Apr 26.
Zettinig G, Weissel M, Flores J, et al. Dermatitis herpetiformis is associated with atrophic but not with goitrous variant of Hashimoto’s thyroiditis. Eur J Clin Invest. 2000;30:53–7.
Hervonen K, Viljamaa M, Collin P, et al. The occurrence of type 1 diabetes in patients with dermatitis herpetiformis and their first-degree relatives. Br J Dermatol. 2004;150:136–8.
Stamnaes J, Dorum S, Fleckenstein B, et al. Gluten T cell epitope targeting by TG3 and TG6; implications for dermatitis herpetiformis and gluten ataxia. Amino Acids. 2010;39:1183–91.
Helsing P, Frøen H. Dermatitis herpetiformis presenting as ataxia in a child. Acta Derm Venereol. 2007;87:163–5.
Hadjivassiliou M, Chattopadhyay AK, Grünewald RA, et al. Myopathy associated with gluten sensitivity. Muscle Nerve. 2007;35:443–50.
Sapone A, Bai JC, Ciacci C, et al. Spectrum of gluten-related disorders: consensus on new nomenclature and classification. BMC Med. 2012;10:13.
Sárdy M, Kárpáti S, Merkl B, et al. Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis. J Exp Med. 2002;195:747–57.
Dieterich W, Laag E, Bruckner-Tuderman L, et al. Antibodies to tissue transglutaminase as serologic markers in patients with dermatitis herpetiformis. J Invest Dermatol. 1999;113:133–6.
Jaskowski TD, Hamblin T, Wilson AR. IgA anti-epidermal transglutaminase antibodies in dermatitis herpetiformis and pediatric celiac disease. J Invest Dermatol. 2009;129:2728–30.
Marietta EV, Camilleri MJ, Castro LA, et al. Transglutaminase autoantibodies in dermatitis herpetiformis and celiac sprue. J Invest Dermatol. 2008;128:332–5.
Preisz K, Sárdy M, Horváth A, et al. Immunoglobulin, complement and epidermal transglutaminase deposition in the cutaneous vessels in dermatitis herpetiformis. J Eur Acad Dermatol Venereol. 2005;19:74–9.
Hall RP, Takeuchi F, Benbenisty KM, et al. Cutaneous endothelial cell activation in normal skin of patients with dermatitis herpetiformis associated with increased serum levels of IL-8, sE-Selectin, and TNF-alpha. J Invest Dermatol. 2006;126:1331–7.
Marietta E, Black K, Camilleri M, et al. A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice. J Clin Invest. 2004;114:1090–7.
Zone JJ, Schmidt LA, Taylor TB, et al. Dermatitis herpetiformis sera or goat anti-transglutaminase-3 transferred to human skin-grafted mice mimics dermatitis herpetiformis immunopathology. J Immunol. 2011;186:4474–80.
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Kárpáti, S. (2015). Dermatitis Herpetiformis. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_44
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DOI: https://doi.org/10.1007/978-3-662-45698-9_44
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