Celiac Disease - NIH Consensus Development Program - National ...

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11. Corrao G, Corazza GR, Bagnardi V, et al. Mortality in patients with coeliac disease and theirrelatives: a cohort study. Lancet. 2001;358:356–361.72
Skin Manifestations of <strong>Celiac</strong> <strong>Disease</strong>John J. Zone, M.D.Dermatitis herpetiformis (DH) is a cutaneous manifestation of celiac disease (CD). Thespectrum of intestinal abnormalities in DH ranges from minimal lymphocyte infiltration of thesmall intestinal epithelium in some patients to complete villous atrophy in others. Only about20 percent of DH patients have intestinal symptoms of CD. Both the skin disease and theintestinal disease respond to gluten restriction and recur with institution of a gluten-containingdiet. It seems likely that the presence of skin disease in DH patients is a marker of CD that isindependent of the severity of the histological CD or the severity of intestinal symptoms. Thecomplications and course of CD, as manifested in DH patients, may represent the true spectrumof CD that would otherwise largely go undiagnosed. Study of DH therefore gives insight into thenature of the spectrum of CD. For this reason, I will review the consensus questions relative toDH and will propose that understanding DH holds the potential to a better understanding of theentire spectrum of CD. (1)How is DH Diagnosed?Three findings support the diagnosis of DH: (1) pruritic papulovesicles on extensorsurfaces; (2) neutrophilic infiltration of the dermal papillae with vesicle formation at thedermal-epidermal junction; and (3) granular deposition of IgA in the dermal papillae of clinicallynormal-appearing skin adjacent to a lesion. The last finding is present in greater than 98 percentof DH patients and is the gold standard for diagnosis. (2) IgA tissue transglutaminase and IgAendomysial antibodies are found in 70–90 percent of patients, indicating that there is apopulation of CD patients who are negative for this serum antibody. IgA antibodies to epidermaltransglutaminase have been proposed as the causative antibody in DH. (3)How Prevalent is DH?DH is most common in those of northern European descent. It is exceedingly uncommonin African Americans and Asians. According to a study in Finland in 1978, the prevalence of DHis 10.4 per 100,000 and the incidence is 1.3 per 100,000. The mean age of onset was in the fourthdecade, but ranged from age 2 to 90. Adolescent and prepubescent children are infrequentlyaffected. This later age of onset is believed to indicate a need for chronic stimulation of themucosal immune system for production of the immune response that causes DH. In contrast toCD in the absence of DH, males outnumbered females 2:1. (4) From 1979 to 1996, the familialincidence of DH was studied prospectively in Finland. DH was diagnosed in 1,018 patients.10.5 percent of patients with DH had one or more affected first-degree relatives. (5)Studies in Utah confirm that DH and familial DH in the United States are of comparableprevalence to European studies. A study of a Utah population reflects the predominant northernEuropean ancestry in that area of the United States. The prevalence of DH in 1987 was 11.2 per100,000. The incidence for the years 1978–1987 was 0.98 per 100,000 per year. The mean age ofonset for males was 40.1 years. The mean age of onset for females was 36.2 years. The male to73
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NIH Consensus Development Conferenc
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III. What Are the Manifestations an
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• What is the management of celia
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Monday, June 28, 2004 (continued)I.
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Monday, June 28, 2004 (continued)II
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Wednesday, June 30, 2004 (continued
Page 13 and 14: Lisa H. RichardsonConsumer Represen
Page 15 and 16: Ciaran P. Kelly, M.D.Director, Celi
Page 17 and 18: Van S. Hubbard, M.D., Ph.D.Director
Page 19 and 20: AbstractsThe following are abstract
Page 21 and 22: susceptibility (e.g., DR17 homozygo
Page 23 and 24: The Pathology of Celiac DiseaseDavi
Page 25 and 26: In this regard, the transport pathw
Page 27 and 28: for the IgG-based test, while speci
Page 29 and 30: 15. de Lecea A, Ribes-Koninckx C, P
Page 31 and 32: Clinical Algorithm in Celiac Diseas
Page 33 and 34: Considera diagnosisof celiac diseas
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Page 37 and 38: Serological Testing for Celiac Dise
Page 39 and 40: Estimates of the sensitivity of the
Page 41 and 42: the risk and severity of CD may als
Page 43 and 44: What Are the Prevalence and Inciden
Page 45 and 46: ascribed to excess menstrual loss.
Page 47 and 48: ReferencesFamily History of Celiac
Page 49 and 50: Carroccio A, Iannitto E, Cavataio F
Page 51 and 52: identified by surveys or through so
Page 53 and 54: Clinical Presentation of Celiac Dis
Page 55 and 56: een widely reported. The question r
Page 57 and 58: The Many Faces of Celiac Disease: C
Page 59 and 60: References1. Green PH, Jabri B. Coe
Page 61 and 62: Association of Celiac Disease and G
Page 63: Does the Gluten-Free Diet Protect F
Page 67 and 68: allow a better understanding of the
Page 69 and 70: 4. Henriksson KG, Hallert C, Walan
Page 71 and 72: characterized, clinically-identifie
Page 73 and 74: 10. Hoffenberg EJ, Emery LM, Barrig
Page 75 and 76: ataxia, epilepsy with posterior cer
Page 77 and 78: Consequences of Testing for Celiac
Page 79 and 80: Osteoporosis/FracturesThere were 11
Page 81 and 82: Dietary Guidelines for Celiac Disea
Page 85 and 86: 21. Thompson T. Thiamin, riboflavin
Page 88 and 89: In order to effectively counsel ind
Page 90 and 91: 9. Hallert C, Granno C, Hulten S, M
Page 92 and 93: adhered to the GFD after more than
Page 94 and 95: Patient education, close supervisio
Page 96: 26. Mustalahti K, Lohiniemi S, Laip
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