American Journal of Clinical Dermatology

, Volume 15, Issue 1, pp 51–56 | Cite as

Dermatitis Herpetiformis: Clinical Presentations Are Independent of Manifestations of Celiac Disease

  • Suneeta Krishnareddy
  • Suzanne K. Lewis
  • Peter H. Green
Original Research Article



Dermatitis herpetiformis (DH) is a skin manifestation of celiac disease (CD), and is often the presenting and only complaint. There are few data comparing those with DH who present only with DH and those with DH who present mainly due to CD.


We compared the prevalence of features usually associated with CD in those who presented with DH with patients in whom DH was part of a typical CD presentation.


A cross-sectional study of a prospectively maintained database of 1,050 patients with CD was analyzed. Only biopsy-diagnosed patients were analyzed for small bowel findings. All patients were included in the analysis of autoimmune diseases and lymphoma incidence. Small bowel biopsies were classified into mild and severe.


The prevalence of villous atrophy was significantly higher in the patients who presented with CD than in those who presented with DH alone (61.8 vs. 12.5 %; p = 0.005). However, the prevalence of nutritional deficiencies, autoimmune diseases, and lymphoma occurred at a similar rate in patients with DH and patients with CD without DH.


Patients who present with CD and concurrent DH are more likely to have more severe pathology than those with predominantly DH, although nutritional deficiencies are similar between the two groups. It is important to screen for nutritional deficiencies in patients with DH, irrespective of the presence of typical CD manifestations.


  1. 1.
    Fry L, Keir P, et al. Small intestinal structure and function and hematological changes in dermatitis herpetiformis. Lancet. 1967;2(7519):729–33.PubMedCrossRefGoogle Scholar
  2. 2.
    Green PH, Cellier C. Celiac disease. N Engl J Med. 2007;357(17):1731–43.PubMedCrossRefGoogle Scholar
  3. 3.
    Zone JJ. Skin manifestations of celiac disease. Gastroenterology. 2005;128(4 Suppl 1):S87–91.PubMedCrossRefGoogle Scholar
  4. 4.
    Fry L, McMinn RM, Cowan JD, et al. Gluten-free diet and reintroduction of gluten in dermatitis herpetiformis. Arch Dermatol. 1969;100:129–35.PubMedCrossRefGoogle Scholar
  5. 5.
    Rubio-Tapia A, Kyle RA, Kaplan EL, et al. Increased prevalence and mortality in undiagnosed celiac disease. Gastroenterology. 2009;137(1):88–93.PubMedCentralPubMedCrossRefGoogle Scholar
  6. 6.
    Rubio-Tapia A, Ludvigsson JF, Brantner TL, Murray JA, Everhart JE. The prevalence of celiac disease in the United States. Am J Gastroenterol. 2012;107(10):1538–44 (quiz 1537, 1545).Google Scholar
  7. 7.
    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;165(2):354–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Fry L, Keir P, McMinn RM, et al. Small-intestinal structure and function and hematological changes in dermatitis herpetiformis. Lancet. 1967;2:729–33.PubMedCrossRefGoogle Scholar
  9. 9.
    Galvez L, Falchuk ZM. Dermatitis herpetiformis: gastrointestinal association. Clin Dermatol. 1991;9:325–33.PubMedCrossRefGoogle Scholar
  10. 10.
    Marsh MN. Studies of intestinal lymphoid tissue. XIII. Immunopathology of the evolving celiac sprue lesion. Pathol Res Pract. 1989;185(5):774–7.PubMedCrossRefGoogle Scholar
  11. 11.
    Gawkrodger DJ, Vestey JP, O’Mahony S, et al. Dermatitis herpetiformis and established coeliac disease. Br J Dermatol. 1993;129:694–5.PubMedCrossRefGoogle Scholar
  12. 12.
    Buckley DB, English J, Molloy W, et al. Dermatitis herpetiformis: a review of 119 cases. Clin Exp Dermatol. 1983;8:477–87.PubMedCrossRefGoogle Scholar
  13. 13.
    Davies MG, Marks R, Nuki G. Dermatitis herpetiformis: a skin manifestation of a generalized disturbance in immunity. Q J Med. 1978;47:221–48.PubMedGoogle Scholar
  14. 14.
    Egan CA, O’Loughlin S, Gormally S, et al. Dermatitis herpetiformis: a review of fifty-four patients. Ir J Med Sci. 1997;166:241–4.PubMedCrossRefGoogle Scholar
  15. 15.
    Thompson T, Dennis M, Higgins LA, Lee AR, Sharrett MK. Gluten-free diet survey: are Americans with coeliac disease consuming recommended amounts of fiber, iron, calcium and grain foods? J Hum Nutr Diet. 2005;18(3):163–9.PubMedCrossRefGoogle Scholar
  16. 16.
    Fry L, Seah PP, McMinn RM, et al. Lymphocytic infiltration of epithelium in diagnosis of gluten-sensitive enteropathy. Br Med J. 1972;3:371–4.PubMedCentralPubMedCrossRefGoogle Scholar
  17. 17.
    Neuhasen SL, Zone JJ, et al. Co-occurrence of celiac disease and other autoimmune diseases in celiacs and their first-degree relatives. J Autoimmun. 2008;313(2):160–5.CrossRefGoogle Scholar
  18. 18.
    Bai D, Brar P, Holleran S, Ramakrishnan R, Green PH. Effect of gender on the manifestations of celiac disease: evidence for greater malabsorption in men. Scand J Gastroenterol. 2005;40(2):183–7.PubMedCrossRefGoogle Scholar
  19. 19.
    Ventura A, Magazzu G, Greco L. Duration of exposure to gluten and risk for autoimmune disorders in patients with celiac disease. SIGEP Study Group for Autoimmune Disorders in Celiac Disease. Gastroenterology. 1999;117(2):297–303.PubMedCrossRefGoogle Scholar
  20. 20.
    Cosnes J, Cellier C, Viola S, et al. Incidence of autoimmune diseases in celiac disease: protective effect of the gluten-free diet. Clin Gastroenterol Hepatol. 2008;6(7):753–8.PubMedCrossRefGoogle Scholar
  21. 21.
    Collin P, Reunala T, Pukkala E, et al. Coeliac disease: associated disorders and survival. Gut. 1994;35:1215–8.PubMedCrossRefGoogle Scholar
  22. 22.
    Christensen OB, Hindsen M, Svensson A. Natural history of dermatitis herpetiformis in southern Sweden. Dermatologica. 1986;173:271–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Weetman AP, Burrin JM, Mackay D, et al. The prevalence of thyroid autoantibodies in dermatitis herpetiformis. Br J Dermatol. 1988;118:377–83.PubMedCrossRefGoogle Scholar
  24. 24.
    Elfström P, Granath F, Ekström Smedby K, Montgomery SM, Askling J, Ekbom A, Ludvigsson JF. Risk of lymphoproliferative malignancy in relation to small intestinal histopathology among patients with celiac disease. J Natl Cancer Inst. 2011;103(5):436–44. doi:10.1093/jnci/djq564.PubMedCrossRefGoogle Scholar
  25. 25.
    Swerdlow AJ, Whittaker S, Carpenter LM, et al. Mortality and cancer incidence in patients with dermatitis herpetiformis: a cohort study. Br J Dermatol. 1993;129:140–4.PubMedCrossRefGoogle Scholar
  26. 26.
    Leslie LA, Lebwohl B, Neugut AI, Gregory Mears J, Bhagat G, Green PH. Incidence of lymphoproliferative disorders in patients with celiac disease. Am J Hematol. 2012;87(8):754–9. doi:10.1002/ajh.23237.PubMedCrossRefGoogle Scholar
  27. 27.
    Malamut G, Chandesris O, Verkarre V, Meresse B, Callens C, Macintyre E, Bouhnik Y, Gornet JM, Allez M, Jian R, Berger A, Châtellier G, Brousse N, Hermine O, Cerf-Bensussan N, Cellier C. Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. Dig Liver Dis. 2013;45(5):377–84. doi:10.1016/j.dld.2012.12.001.PubMedCrossRefGoogle Scholar
  28. 28.
    Hervonen K, Alakoski A, Salmi TT, Helakorpi S, Kautiainen H, Kaukinen K, Pukkala E, Collin P, Reunala T. Reduced mortality in dermatitis herpetiformis: a population-based study of 476 patients. Br J Dermatol. 2012;167(6):1331–7.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2013

Authors and Affiliations

  • Suneeta Krishnareddy
    • 1
  • Suzanne K. Lewis
    • 1
  • Peter H. Green
    • 1
  1. 1.Celiac Disease Center, Department of MedicineColumbia University College of Physicians and SurgeonsNew YorkUSA

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