Dental Problems and Oral Manifestations of Celiac Disease

Dental Problems and Oral Manifestations of Celiac Disease

Widodo Judarwanto. Picky Eaters and Grow Up Clinic, Jakarta Indonesia

Celiac disease is a common disorder affecting both children and adults. As many people with celiac disease do not present with the classic malabsorptive syndrome, delays in diagnosis are common. Dental enamel defects and recurrent aphthous ulcers, which may occur in patients with celiac disease, may be the only manifestation of this disorder. When dentists encounter these features, they should enquire about other clinical symptoms, associated disorders and family history of celiac disease. In suspected cases, the patient or family physician should be advised to obtain serologic screening for celiac disease and, if positive, confirmation of the diagnosis by intestinal biopsy. Dentists can play an important role in identifying people who may have unrecognized celiac disease. Appropriate referral and a timely diagnosis can help prevent serious complications of this disorder. The higher prevalence of recurrent aphids stomatitis in celiac disease led us to investigate the celiac disease prevalence in patients with recurrent aphthous stomatitis, which might assist in diagnosis of asymptomatic celiac disease patients. It is important to identify this disease process early because affected individuals have an increased risk for developing lymphoma of the gut.

Celiac disease (CD) is a condition related to the small intestine’s intolerance to gluten. In epidemiologic studies the prevalence is highly variable. The diagnosis can be difficult due to the wide spectrum of signs and symptoms. With a prevalence of 1% in western populations, Celiac disease (CD) is one of the most common inflammatory disorders of the small intestine. CD is often assumed to have its onset in childhood, but it has recently been suggested that adults can also develop CD. Clinical manifestations vary according to age group: infants and young children present with diarrhea, abdominal distention, and failure to thrive, whereas adults that develop CD not only present with diarrhea, but also with silent manifestations such as anemia, osteoporosis, or neurological symptoms. In the small intestine of celiac disease patients, dietary wheat gluten and similar proteins in barley and rye trigger an inflammatory response. While strict adherence to a gluten-free diet induces full recovery in most patients, a small percentage of patients fail to recover.

As the risk for intestinal lymphoma is higher in these patients, early diagnosis has its privileges. The celiac disease (CD) may be correlated with the insurgence of some oral signs, as based on a systematic review of the literature. Should this correlation be proven, any dentistry’s screening would then be important to diagnose early celiac sprue pathogenesis. A literature survey was accomplished by using the Medline database (Entrez PubMed). The survey, which covered the period from the year 1972 to 2009, provided 382 published articles. Of these, 29 articles were selected according to inclusionary/exclusionary criteria, and consequently qualified for the final review analysis. Manual searching through the reference lists of the selected articles allowed the obtainment of others 17 articles. Findings gathered through this literature’s review corroborate the significance of a causal relationship between some oral signs and CD. There are enough evidence making the correlation between CD and oral defects scientifically sustainable. This recognition should lead dentists to play more significant roles in screening for CD, as otherwise, if not properly diagnosed and not treated with a gluten-free diet, may eventually cause some malignancies.

Enamel defects and recurrent aphthous ulcers are common manifestations of celiac disease. Increased awareness of this disorder, along with careful questioning about other symptoms, family history, serologic screening tests and appropriate referral can help establish a timely diagnosis and prevent complications of untreated celiac disease. The diagnosis of celiac disease can sometimes be made from a smile!

The dramatic improvement in knowledge concerning celiac disease (CD) has disclosed the pattern of the associated clinical manifestations and the often atypical or silent presentation of this disease, which makes clinical diagnosis difficult. Also oral manifestations, mostly recurrent apthous stomatitis (RAS) and dental enamel hypoplasia, are atypical signs of CD. Our opinion about the possibility of performing mass-screening to reveal atypical or silent CD is in agreement whit who is asserting that a sistematical case-finding is, at present, the most suitable epidemiological approach. So, we think that patients affected by RAS, or dental enamel hypoplasia, should be considered, even in the absence of any gastrointestinal symptom, at-risk subjects, and should therefore undergo diagnostic procedure for CD.

Patients with systematic dental enamel defects should be screened for CD even in the absence of gastrointestinal symptoms. CD screening tests for patients with oral aphthae or idiopathic atrophic glossitis should be selectively considered during a medical evaluation that focuses on all aspects of the patient’s status.

Oral Manifestations

In a subset of these refractory celiac disease patients, an (aberrant) oligoclonal intraepithelial lymphocyte population develops into overt lymphoma. Celiac disease is strongly associated with HLA-DQ2 and/or HLA-DQ8, as both genotypes predispose for disease development. mmunohistochemistry of the small intestine of patients shows villous atrophy, crypt hyperplasia, and elevated levels of intraepithelial lymphocytes (IELs). The only therapy until now is a gluten-free diet, which will normalize the clinical and histological manifestations and allows the patients to live an otherwise normal life. part of the symptoms are oral manifestations as dental enamel defects, aphthous ulcers and Atrophic Glossitis. The prevalence of caries in CD patiens is law as compared to the healthy population and in some cases the normal eruption sequence of the teeth was damaged. Part of the undiagnosed CD patients are among our patients and the enamel defects they present are misdiagnosed as tetracycline pigmentation or white spot lesions it is the practitioners responsibility to add CD as a possible cause to the findings and refer the patient to further examination. The case report in this paper presents a 47 years old female patient who was a patient in my practice for 25 years. When she became 40 she was diagnosed as CD and treated accordingly over the years enamel defects were treated conservatively. As the lesions became more frequent and unpredictable the patient demanded a full mouth solution that would restore both the functional and esthetical problems she had with her dentition. A full Porcelane Fused to Metal (PFZ) crowns were fabricated and the patient got a solution to both here demands. The crowns used were 3M Lava zirconia crowns and the case is presented as step by step sequence.

Dental Problems

Avsar reported that investigate the presence and distribution of developmental enamel defects and caries in children with celiac disease (CD) and compare the results obtained with those of a control group of children without CD. A total of 64 subjects selected from patients diagnosed and treated for CD were studied. Sixty-four age/ sex-matched healthy children were enrolled as a control group. Permanent dentition enamel defects were recorded according to Aine’s classification. The caries experience of the children was recorded according to the criteria of the World Health Organization (WHO). The prevalence of enamel defect in CD subjects was found to be significantly higher than in healthy subjects. Grade I type enamel defects were most commonly diagnosed in both groups. The number of caries-free subjects in the control group was higher than in the CD group. CD were at an increased risk of dental enamel defects compared with healthy subjects. Enamel defects were associated with an increased caries incidence.

Systematic and chronologically distributed permanent-tooth so-called coeliac-type enamel defects are highly prevalent both in children and adults with coeliac disease (gluten-sensitive enteropathy) and dermatitis herpetiformis. Coeliac-type enamel defects were also found in healthy first-degree family members of coeliac disease patients. Our family study showed that these persons with the typical defected enamel were genetically similar to coeliac disease patients (A1;B8;DR3). As coeliac disease patients are often clinically silent with no gastrointestinal symptoms, or they complain only of minimal abdominal discomfort, both dentists and physicians could select patients with coeliac-type enamel defects for gastroenterological and dermatological consultations, including serological screening tests and later jejunal mucosal biopsy.

Celiac disease can develop at any age when solid foods are introduced into the diet; however, if it appears in children while the permanent teeth are developing, i.e., before 7 years of age, abnormalities in the structure of the dental enamel can occur. These defects are seen most commonly in the permanent dentition and tend to appear symmetrically and chronologically in all 4 quadrants, with more defects in the maxillary and mandibular incisors and molars. Both hypoplasia and hypomineralization of the enamel can occur. A band of hypoplastic enamel, often with intact cusps, is common. A hiatus in enamel and dentin formation can occur at a developmental stage corresponding to the onset of gastrointestinal symptoms. Dental enamel defects are common in children who develop symptoms of celiac disease before 7 years of age. Such defects are not seen as frequently in adults with celiac disease, as they may have developed symptoms at a later age or have had severely affected abnormal teeth altered or extracted.

The exact mechanism leading to these defects is not clear, but immune-mediated damage is suspected to be the primary cause.Nutritional disturbances, including hypocalcemia, may also play a role. Stimulation of naïve lymphocytes by gluten in the oral cavity has also been hypothesized.

The overall prevalence of systemic dental enamel defects in celiac disease patients with mixed or permanent dentition ranges from 9.5% to 95.9% (mean 51.1%); in patients with deciduous teeth, prevalence is 5.8% to 13.3% (mean 9.6%). This difference can be explained by the fact that the crowns of permanent teeth develop between the early months of life and the seventh year (i.e., after the introduction of gluten in the diet) whereas the development of deciduous teeth occurs primarily in utero. The involvement of deciduous teeth in some cases supports the hypothesis that immunologic and genetic factors are more important in the etiology of the defects than nutritional deficiencies. Dental enamel defects are also found in healthy first-degree relatives of patients with celiac disease, further supporting an immunogenetic basis for causation.

Enamel defects include pitting, grooving and sometimes complete loss of enamel. A classification of these defects in celiac disease was developed by Aine and colleagues7 (Table 1). The various grades of defects are illustrated in Figs. 1, 2 and 3.

The prevalence of dental caries in children with celiac disease varies. It was found to be no different than the general population in 1 study8 but higher in another.11 However, in both these studies, dental enamel defects were more common in patients with celiac disease compared with controls.

Recurrent aphthous ulcers can also occur in celiac disease and may provide another clue to the possible presence of the disorder (Fig. 4). In a large survey of a Canadian population with biopsy-proven celiac disease, 16% of children (< 16 years of age) and 26% of adults reported having recurrent oral ulcers.The exact cause of aphthous ulcers in celiac disease is unknown; however, it may be related to hematinic deficiency, with low serum iron, folic acid and vitamin B12 due to malabsorption in patients with untreated celiac disease.

Figure 4: Aphthous ulcers in the buccal mucosa.

As enamel defects can be seen in several disorders, other possible etiologic factors should be considered before celiac disease can be firmly established as the principle cause (Table 2). The dentist can readily determine whether an etiologic factor is systemic, affecting all teeth developing at the same time (e.g., the anterior incisors and the first molars), or local, affecting only 1 or 2 teeth. Enamel hypoplasia is easily identified, as parts or all of the enamel can be poorly formed or missing altogether (Table 2, rows A, C). Localized enamel hypoplasia can take the form of pitting, grooves or deep lines across the facial surfaces, which can be normally mineralized (shiny and hard). An intact, smooth enamel surface with opaque white or yellow spots or lines indicates a less-severe hypocalcified disturbance. Dental fluorosis (Table 2, row B) is a common enamel defect.18 Amelogenesis imperfecta (Table 2, row C) is a relatively rare disorder, usually characterized by enamel pitting but sometimes appearing as hypocalcification.19 When some enamel appears to be normal, a history of vitamin D deficiency,16 severe infections or prematurity20 should be explored. When the child recovers from these systemic problems, enamel formation returns to normal.

Enamel hypoplasia as  clinical marker of early diagnosis.

 Bossù M et al reported study that assess at the scansion electron microscope (SEM) the structural aspects of enamel hypoplasia (EH) in coeliac disease (CD) with the aim to investigate our hypothesis of a possible significant difference between structural characteristics of EH in CD affected patients and EH of non-coeliac patients. If the presence of specific features of the EH associated with CD were demonstrated, these findings would represent for the dentist early non-invasive clinical markers of diagnosis of CD in case of suspected disease.

Bossù M analysed at SEM two samples of enamel fragments from hypoplasic teeth, both deciduous and permanent, harvested from 10 coeliac children (18 permanent teeth, 6 deciduous teeth; study group) and 10 non-coeliac children (16 permanent teeth, 4 deciduous teeth; control group) treated for dental caries, dental extractions for extensive caries lesions or deciduous teeth exfoliation.

Significant structural differences were noted between EH of non-coeliac patients and same dental ? lesion in the group with CD. In the study group, EH defects were predominantly located on the central and lateral incisors, upper and lower, both deciduous and permanent, and on the first permanent molars, and were always simmetrical. EH of permanent teeth of CD affected patients was characterised by prisms more irregularly distributed with irregular margins and less interprismatic substance than observed in non-coeliac EH. The deciduous teeth of the study group showed shorter enamel prisms with a non-parallel direction up to convergence and less interprismatic substance than observed in the control group.

This morphological analysis at SEM of the hypoplasic enamel defects of a group of coeliac children, the first published in literature, demonstrates that the EH of deciduous and permanent teeth in CD is highly hypomineralised with shorter prisms, more irregularly distributed and less interprismatic substance than observed in the non-coeliac EH. More data are needed to validate the significance OF our observations with the aim to assess if this simple, non-invasive microscopic analysis can be considered effective for the early identification of silent cases of CD that otherwise would not be diagnosed in the paediatric age.

Recurrent aphthous stomatitis.

Recurrent aphthous stomatitis is a common disease of the oral mucosa that is characterized by recurrent, painful ulcers of unknown etiology. The association between celiac disease and recurrent aphthous stomatitis has been evaluated in several studies, but variable results have been reported. Yaşar S, et al. reported study that determine the prevalence of celiac disease in patients with recurrent aphthous stomatitis. Methods: The study group consisted of 82 patients, all of whom had a history of recurrent aphthous stomatitis. The control group included 82 patients who did not have aphthous stomatitis. Patients were screened for IgA anti-endomysial antibodies, IgG anti-endomysial antibodies, IgA anti-gliadin antibodies, and IgG anti-gliadin antibodies. Patients with positive serology underwent endoscopic biopsies of the duodenal mucosa. Patients in both groups were also questioned regarding gastrointestinal symptoms.

There is 1.2 percent of the study group was diagnosed with celiac disease by biopsy. Gastroesophageal reflux disease symptoms, heartburn and regurgitation were determined to be of higher incidence in the study group. None of the 82 patients in the control group were diagnosed as having celiac disease. There is no apparent etiological link between recurrent aphthous stomatitis and celiac disease and that screening recurrent aphthous stomatitis patients for celiac disease has little clinical value. Additionally, regurgitation of gastric acid to the oral cavity may precipitate the formation of aphthous stomatitis.

The study group consisted of patients having a history of recurrent aphthous stomatitis. The control group included patients not having aphthous stomatitis. Antibodies to gliadin IgG and IgA and antibodies to endomysium were determined from the serum samples of all patients. Biopsies were obtained from the distal part of the duodenum.

Biopsies of two patients (4.8%) out of 41 belonging to the study group were diagnosed as celiac disease. In serum samples of both, antibodies to gliadin IgA and antibodies to endomysium were found to be positive. Antibodies to gliadin IgG antibody were positive in only one of these two patients. None of the 49 patients in the control group was diagnosed as celiac disease.

Further evaluation of recurrent aphthous stomatitis patients for celiac disease must be performed. As the endoscopic procedures are invasive and costly, evaluation of recurrent aphthous stomatitis patients for celiac disease must include serologic markers at the beginning. If any positivity is determined in markers, then endoscopic procedures including biopsies of the duodenum must be considered as the second-step intervention.

Sedghizadeh PP, et al.also reported that evaluate whether patients with CD have a significantly higher prevalence of recurrent aphthous stomatitis compared with the general population, as some investigators have speculated. Therefore, these study screened 61 patients with diagnosed CD for the presence of, or a positive history of, aphthous ulcerations. Statistically compared this data with a randomly selected control population, matched for age and gender, but without CD. These study results demonstrated no significant differences between groups for age, gender, or prevalence of recurrent aphthous stomatitis.

Recurrent aphthous stomatitis is a common disease of the oral mucous membranes. Currently a hypothesis is being discussed that it might be pathogenetically related to coeliac disease. The frequency of coeliac disease anti-endomysial (or anti-transglutaminase) antibodies in patients with recurrent aphthous stomatitis. Blood samples from 42 patients were evaluated and 4.7% were IgA- and IgG-endomysial antibody-positive. None of the 42 persons in the control group had antibodies, which was not statistically different from the patient group. The two antibody-positive patients had episodes of mild gastrointestinal symptoms only, but histopathology of duodenal mucous membranes confirmed coeliac disease. All symptoms related to aphthous stomatitis responded well to a gluten-free diet. Every patient with recurrent aphthous stomatitis should be asked about a history of gastrointestinal complaints and screened for markers of coeliac disease, since recurrent aphthous stomatitis may in some cases respond to a gluten-free diet.


  • Rashid M, Zarkadas M, Anca A, Limeback H. Oral manifestations of celiac disease: a clinical guide for dentists. Can Dent Assoc. 2011;77:b39.
  • Pastore L, De Benedittis M, Petruzzi M, Tatò D, Napoli C, Montagna MT, Catassi C, Serpico R. Importance of oral signs in the diagnosis of atypical forms of celiac disease. Recenti Prog Med. 2004 Oct;95(10):482-90.
  • Giuca MR, Cei G, Gigli F, Gandini P. Oral signs in the diagnosis of celiac disease: review of the literature. Minerva Stomatol. 2010 Jan-Feb;59(1-2):33-43.
  • Cheng J, Malahias T, Brar P, Minaya MT, Green PH. The association between celiac disease, dental enamel defects, and aphthous ulcers in a United States cohort. J Clin Gastroenterol. 2010:44(3):191-4.
  • Avşar A, Kalayci AG. The presence and distribution of dental enamel defects and caries in children with celiac disease. Turk J Pediatr. 2008 Jan-Feb;50(1):45-50.
  • Pastore L, Carroccio A, Compilato D, Panzarella V, Serpico R, Lo Muzio L. Oral manifestations of celiac disease. J Clin Gastroenterol. 2008;42(3):224-32.
  • Pastore L, Campisi G, Compilato D, Lo Muzio L. Orally based diagnosis of celiac disease: current perspectives. J Dent Res. 2008;87(12):1100-7.
  • Fraser D, Nikiforuk G. The etiology of enamel hypoplasia in children — a unifying concept. J Int Assoc Dent Child. 1982;13(1):1-11.
  • Aine L.  Coeliac-type permanent-tooth enamel defects. Ann Med. 1996 Feb;28(1):9-12.
  • Sedghizadeh PP, et al. Celiac disease and recurrent aphthous stomatitis: a report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002 Oct;94(4):474-8.
  • Yaşar S, et al. Clinical importance of celiac disease in patients with recurrent aphthous stomatitis. Turk J Gastroenterol. 2012 Feb;23(1):14-8.
  • Celiac disease in patients having recurrent aphthous stomatitis. Turk J Gastroenterol. 2004 Sep;15(3):192-5.
  • Aydemir S, et al. Celiac disease in patients having recurrent aphthous stomatitis. Turk J Gastroenterol. 2004 Sep;15(3):192-5
  • Olszewska M, et al.Frequency and prognostic value of IgA and IgG endomysial antibodies in recurrent aphthous stomatitis. Acta Derm Venereol. 2006;86(4):332-4.
  • Bossù M, Bartoli A, Orsini G, Luppino E, Polimeni A.  Enamel hypoplasia in coeliac children: a potential clinical marker of early diagnosis. Eur J Paediatr Dent. 2007 Mar;8(1):31-7.




Pediatric Articles Dr Widodo Judarwanto (pediatrician)

100 Favorites Articles for Professional



Provided By JURNAL PEDIATRI ONLINE Address: Jl Matraman 30 Jakarta Pusat 10430 phone 021-29614252 – 08131592-2012 – 08131592-2013. “Children are the world’s most valuable resource and its best hope for the future”. We are guilty of many errors and many faults. But our worst crime is abandoning the children, neglecting the fountain of life. In 1,000 days Your Children, You can change the future

Information on this web site is provided for informational purposes only and is not a substitute for professional medical advice. You should not use the information on this web site for diagnosing or treating a medical or health condition. You should carefully read all product packaging. If you have or suspect you have a medical problem, promptly contact your professional healthcare provider

Copyright ©2016, Jurnal Pediatri Online, Information Education Networking. All rights reserved

One response to “Dental Problems and Oral Manifestations of Celiac Disease

Tinggalkan Balasan

Isikan data di bawah atau klik salah satu ikon untuk log in:


You are commenting using your account. Logout /  Ubah )

Foto Google

You are commenting using your Google account. Logout /  Ubah )

Gambar Twitter

You are commenting using your Twitter account. Logout /  Ubah )

Foto Facebook

You are commenting using your Facebook account. Logout /  Ubah )

Connecting to %s