Article ID: WMC004448 ISSN 2046-1690

Major dental clinical pathological manifestation of celiac disease

Peer review status: No

Corresponding Author: Dr. Andrea Quaranta, dentist, Dipartimento di Scienze Odontostomatologiche e Maxillo Facciali, Universita' degli Studi di Roma - Italy

Submitting Author: Dr. Francesca Muggiano, Dentist, Dipartimento di Scienze Odontostomatologiche e Maxillo Facciali, Universita' degli Studi di Roma - Italy

Other Authors: Dr. Ivana Giannantoni, dentist, Dipartimento di Scienze Odontostomatologiche e Maxillo Facciali, Universita\' degli Studi di Roma - Italy

Article ID: WMC004448 Article Type: Review articles Submitted on:22-Nov-2013, 11:33:42 AM GMT Published on: 23-Nov-2013, 05:43:50 AM GMT Article URL: http://www.webmedcentral.com/article_view/4448 Subject Categories:ORAL MEDICINE Keywords:Celiac disease, oral medicine, oral pathology, enteropathy, recurrent aphthous stomatitis, atrophic glossitis, oral Lichen Planus, Sjogren disease, dermatitis herpetiformis, dental enamel hypoplasia How to cite the article:Muggiano F, Quaranta A, Giannantoni I. Major dental clinical pathological manifestation of celiac disease. WebmedCentral ORAL MEDICINE 2013;4(11):WMC004448 Copyright: This is an open-access article distributed under the terms of the Creative Commons Attribution License(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Source(s) of Funding: No source of funding.

Competing Interests: No competing interests.

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Major dental clinical pathological manifestation of celiac disease

Author(s): Muggiano F, Quaranta A, Giannantoni I

Abstract enzyme transglutaminase. Subsequently glydin binds to HLA DQ2 and DQ8 portion of antigen presenting cells and activates cd4 t lymphocytes found in the lamina propria intestine mucosa. Celiac disease or sprue is an autoimmune disorder that leads the blunting and loss of villi of the small Tissutal damages are hence caused by immune intestine due to the gluten assumption which is a system cross-reaction that produces anti-gliadin proteic complex found in various cereal product. antibodies and antibodies direct to tissue Celiac symptoms are mostly various: diarrhoea, failure transglutaminase and tTG-gliadin proteic complex. to gain weight(in young children), reduction of adipose B cell lymphocytes are also involved in the tissue, irritability are the most common symptoms, pathogenesis producing anti-giadin, anti-endomysium though other signs and symptoms be clinically Ig A and anti transglutaminase antibodies. In celiac suggestive of celiac disease. Among the most disease the oral cavity is also subjected to tissue characteristic symptoms there are migraine, iron alterations that indicate diagnostic suspect. Therefore deficiency anaemia, hypertransaminasemia, an accurate clinical exam of the oral cavity allows to hepatosteatosis, meteorism. Other clinical symptoms reveal soft and hard tissue lesions associated to celiac related to the oral cavity but not properly disorders. pathognomonic of, can be observed in all clinical Clinical expressions associated to celiac disorders forms of celiac disease and suggest clinicians to located in the oral cavity are: require more specific diagnostic analysis and exams. Recurrent Aphthous Stomatitis Introduction Recurrent Aphthous Stomatitis (RAS) results to be the most frequent pathological expression that concerns the oral cavity. There are three different clinical forms Celiac disease is a chronic enteropathy autoimmune of RAS: minor aphthous ulcer, major aphthous ulcer disorder that affects genetically predisposed and herpetiform aphthous ulcers. individuals of both gender at any age, but has a The common features are the ulcerative lesions of female prevalence of the sex ratio. It is an oral mucosa that arise with a burning sensation and inflammatory disease that concerns tenuous intestine progressively the affected sites become mucosa, leading to a progressive intestine villi erythematosus and evolves in erosive lesions: flattening and their eventually complete atrophy causing nutritive substances malabsorption. Minor aphthous ulcers: (diameter inferior to 1 cm) clinically appear as roundish ulcer with well defined Matherials and methods border and a yellowish fibrous bottom, surrounded by an erythematosus halo. They are elective for non keratin mucosa sites, specifically for the buccal Etiopathogenesis mucosa of the lower lip . Celiac disorder affects genetically predisposed to Major aphthous ulcers (diameter superior to 1 cm) gliadin intolerance individuals. Glydin is a gluten have a longer clinical progress compared to minor proteic fraction that causes an intestine inflammatory aphthous ulcer. They appear with an irregular reaction and the afterwards villous atrophy. In crateriform shape and well-defined, infiltrated physiological conditions intestine epithelium, thanks to prominent border surrounded by a peripheral edema; intercellular tight junctions, forms a barrier to the the lesion bottom is rich of fibrous greyish tissue. passage of several macromolecules as gluten. In Often they are associated to halitosis, hypersalivation, celiac disorders such barrier is altered. Therefore dysphagia, dysphonia, fever, feeling of general glydin overcome the low bowel mucosal barrier and discomfort, localized lymphadenopathy and the most gets in contact with the lamina propria mucosa where frequent affected sites are the labial mucosa, palatine it interacts with and is structurally modified by the arches and soft palate.

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Herpetiform aphthous ulcers appears as numerous erythematosus and ulcerative areas surrounded at the groups of punctiform ulcerations (ranging from 10 to borders by reticular striae. Gingival ulcerative lesions 100 elements and with dimensions inferior to 3mm). mostly need an histopathological corroboration since it This latter group prevails in the female sex and in can simulate other diseases such as pemphigus adults, often it is associated dysphonia, dysphagia and vulgaris, benign mucous membrane pemphigoid or hypersalivation. else evolves in oral carcinoma. The possible connection with the celiac disease is Plaque-like lichen is usually observed all over the supported by the complete remission of the oral oral cavity and clinically appears as irregular and aphthous ulcers in patient receiving a gluten free diet translucent white plaque surrounded by an and by the recurrence of aphthous lesions after the erythematosus halo. introduction of gluten in ordinary diet. Pigmentotous Lichen is a very rare form Clinically celiac patients show most frequently minor characterized by the presence of pigmented papulae aphthous ulcers. derived from in addition to the regular white lesions. Atrophic glossitis Sjogren disease Glossitis is an inflammation of the lingual mucosa; in Sjogren disease is an autoimmune disease celiac disorders, the tongue initially appear swollen, characterized by the progressive destruction of flushed and de-ephithelialized and it is associated to a exocrine glands associated to other autoimmune sensation of dryness, burning and pain during manifestations. There are two clinical variants: a swallowing. Later on the tongue become lucid, smooth primitive form (where the clinical manifestations are and pale due to the papillae atrophy. The B12 vitamin exclusively concerning the exocrine system) and a deficiency due to celiac nutritional malabsorption is the secondary form (where other systemic tissues are origin of this pathological expression. involved in particular the connective tissue). The Oral Lichen Planus disease can debut with mucosal dryness or with an aspecific symptomatology. The major clinical Oral Lichen Planus is a mucocutaneous disease, that expression in the oral cavity of the Sjogren disease is affect both skin and mucosal sites as oral cavity, represented by dryness of mouth (xerosthomia) due to vagina penis esophagus. Lichen Planus affects skin a reduced secretive function of salivary glands. (in particularly at the flexor surface of limbs) as small Patients can have problems swallowing and a burning purple polygonal pruritic papulae; Lichen Planus can sensation, they also have a greater predisposition to affect also scalp and hair follicles. It can also involves dental caries and the placement of prosthetic devices all the surfaces of the oral cavity specifically the cheek are troublesome. Clinical physical exam of the oral mucosa, bilaterally, tongue and gums. Possibly celiac mucosa shows dryness, hyperemia and filiform disorders and Lichen Planus have the same auto papillae atrophy. Salivary glands appear hard movable, immune etiopathogenetic mechanisms. multilobulated and asymmetric. Another useful Oral Lichen Planus when affects oral mucosa can diagnostic sign is xerophtalmia, which is a defective have variable clinical onset and forms: function of lachrymal excretion that causes Reticular Lichen has the classic clinical aspect of photosensitivity, burning sensation and conjunctivitis net-like Wickham's striae observed usually at the symptoms. Autoimmune manifestations of Sjogren posterior buccal fornix. Other sites are, decreasing disease are similiar to those that carctherized celiac frequency, gums, lingual dorsum and edges, palate disease. and lips. A peculiar feature of oral Lichen Planus Dermatitis Herpetiformis which important for the differential diagnosis is the Dermatitis Herpetiformis or Duhring's disease is a tendency of affecting both sides, bilaterally. cutaneous pathology autoimmune based, strictly Bullous Lichen shows blisters containing serum that related to celiac disease. It shows erythematosus and easily get broken leaving and ulcerative lesion area. urticaria cutaneous areas with very pruriginous Atrophic Lichen is located on the lingual dorsum and blisters and vesicles at the periphery. In the oral cavity causes lingual papillae atrophy associated to white it express itself with erythematosus lesions that later plaques or reticular striae. Almost in all cases acid evolve in vesicles whose breakage provoke painful and hot food arise painful and burning sensations. and sometime bleeding erosions. Erosive/Ulcerative Lichen is mainly observed on Dental Enamel Hypoplasia buccal lingual and gingival mucosa and appears as Enamel Hypoplasia is a qualitative and quantitative

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alteration of dental hard mineralized tissues, due to condition ,which generally need a multidisciplinary an altered mineralization process of the dental enamel specialist approach reducing and prevent pathological matrix. drawbacks of unthreatened celiac patients on other The determining factor for the onset of enamel organs. demineralization defects in celiac patients is unknown References but the most likely hypothesis is either the hypocalcemia related to calcium malabsorption in the small bowel portion of the intestine during 1. Report of Working Group of European Society of amelogenesis or the autoimmune mediating Paediatric Gastroenterology and Nutrition. Revised lymphocyte response versus the enamel organ. criteria for diagnosis of coeliac disease. Arch Dis Child Some authors have suggested that both the 1990;65:909-11. etiopathogenetic mechanisms are synergistically 2. Ellis HJ, Pollock EL, Engel W, Fraser JS, involved in dental tissue alterations onset. Rosen-Bronson S, Wieser H, Ciclitira PJ. Investigation Enamel lesions have different pattern ranging from of the putative immunodominant T cellepitopes in singular discoloration to evident dental shape and coeliac disease. Gut. 2003 Feb;52(2):212-7. color anomalies. 3. Ascher H., Krantz I. & Kristiansson B. Increasing incidence of coeliac disease in Sweden. Arch Dis Nowdays enamel lesions found in celiac individuals Child, 66, 608. 1991 are classified on the Aine classification system (1986): 4. Barker JM, Liu E . Celiac disease: pathophysiology, Grade 0: absence of defects. clinical manifestations, and associated autoimmune Grade 1: cromatic alterations: single or multiple yellow conditions. Adv Pediatr 55: 349–65. 2008 or brown opaqueness with well-defined or blurred 5. Sollid LM. Molecular basis of celiac disease. Ann borders. absence of enamel brightness. Rev Immunol 2000;18:53-81 6. Aguirre,Rodriguez,Oribe,Vitoria,:Dental enamel Grade 2: mild structural defects: rough surface defects in coeliac patients.oral surg oral med oral path covered by orizzontal furrows or superficial cavity. oral endod 1997;84:646-50. Discoloration and opacity can be present.a portion or 7. Petrecca,Giammaria,Giammaria:Modificazioni del the whole enamel surface has low brightness. cavo orale nel bambino affetto da malattia Grade 3: evident structural defects: a portion or the celiaca,Minerva Stomatol 1994,43:137-40. whole enamel surface is rough and has deep 8. orizzontal furrows that have different width or wide Bertoldi,Balli,Tanza;Bertolani,Chiarini:Sperimentazioni cavity. variable opaque ares are present. ed analisi clinica dei rapporti intercorrenti tra il danno Grade 4: severe structural defects: dental morphology dentale e la malattia celiaca. Minerva is subverted,cuspidal margins are sharp whereas Stomatol1995;44:95-105; incisal margins are rough and thinned.serious 9. Greco L, Auricchio S, Mayer M, Grimaldi M. Case substantial discoloration. Other distictive signs of control study on nutritional risk factors in celiac celiac disease are the symmetric and bilateral dental disease. J Pediatr Gastroenterol Nutr 1988;7: 395-9 lesions occurring in various tooth of both the arches 10. Fasano A. Surprises from celiac disease. Sci Am and the chronological coherence of the lesions arisen 2009;301:54-61. during amelogenesis. 11. Walker-Smith JA, Guandalini S, Schmitz J,et al. Table I Pathological manifestation with high/low Revised criteria for diagnosis of coeliac disease. Arch association grade. Dis Child 1990;65:909–11. 12. Fasano A, Catassi C. Current approaches to Conclusions diagnosis and treatment of celiac disease: an evolving spectrum. Gastroenterology 2001; 120: 636-51. 13. Di Sabatino A, Corazza GR. Coeliac disease. During internal and external clinical exam of the oral Lancet. 2009;373:1480-93. cavity, clinicians can detect the previously exposed 14. Fasano A, Catassi C. Coeliac disease in children. pathological oral manifestations and advance a valid Best Pract Res Clin Gastroenterol. 2005;19(3):467-78 diagnostic suspect for celiac disease in those patients 15. Ficarra G, Manuale di patologia e medicina orale where this lesions are traced out. When those clinical 3° ed. The McGraw-Hill Companies 10-2006. manifestations appears in childhood, they can help to 16. Pindborg Jens J. Atlante delle malattie della early diagnosed and therapeutically treat such mucosa orale ed. Elsevier 1995.

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17. J. V. Soames, J. C. southham. Patologia Orale 3° ed. 2005. 18. Valletta, Matarasso, Vignogna; Malattie odontostomatologiche ed. 2005. 19. Harrison. Principi di medicina interna. The McGraw-Hill Companies ed. 2007. 20. Amerio P.L., Bernengo M.G., Calvieri S., Chimenti S., Pippione M., Aricò M., Aste N., Borroni G., Leigheb G., Micali G., Nunzi E., Offidani A.M., Tulli A. Dermatologia e Venereologia. Minerva Medica. Ed. 2009. 21. Roversi. Manuale di diagnostica e terapia. Merqurio editore ed. 2012.

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Illustrations

Illustration 1

Fig. 1 Vestibular Major Aphthous Ulcer.

Illustration 2

Fig. 2 Underlip Minor Aphthous Ulcer.

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Illustration 3

Fig. 3 Herpetiform Stomatitis.

Illustration 4

Fig. 4 Oral manifestation of Herpetiform Dermatitis.

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Illustration 5

Fig. 5 Oral Lichen Planus.

Illustration 6

Fig. 6 Dental Enamel Hypoplasia.

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Illustration 7

Table I. Pathological manifestation with high/low association grade.

Pathological manifestation with high Pathological manifestation with low association grade association grade Stomatite aftosa ricorrente Jawbones development delay

Atrophic Glossitis Dental eruption delay

Oral Lichen planus Aggressive dental caries

Sjogren disease Candidiasis

Dermatitis herpetiform

Enamel hypoplasia

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