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Oral Manifestations of Systemic Diseases: Overview, Gastrointestinal Diseases, Nutritional Disease 12/09/16, 11:27

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Oral Manifestations of Systemic Diseases: Overview, Gastrointestinal Diseases, Nutritional Disease 12/09/16, 11:27 Oral Manifestations of Systemic Diseases: Overview, Gastrointestinal Diseases, Nutritional Disease 12/09/16, 11:27 Oral Manifestations of Systemic Diseases Author: Heather C Rosengard, MPH; Chief Editor: Dirk M Elston, MD more... Updated: Jul 27, 2016 Overview The oral cavity plays a critical role in numerous physiologic processes, including digestion, respiration, and speech. It is also unique for the presence of teeth and mucosa. The mouth is frequently involved in conditions that affect the skin, but it is also affected by many systemic diseases. Oral involvement may precede or follow the appearance of findings at other locations. This article is intended as a general overview of conditions with oral manifestations of systemic diseases. It is not intended to provide details about the diagnosis and management of these conditions. Many of these conditions have excellent full- length Medscape Drugs & Diseases articles, which are linked herein. Gastrointestinal Diseases The oral cavity is the portal of entry to the GI tract and is lined with stratified squamous epithelium. The oral cavity is often involved in conditions that affect the GI tract. Both ulcerative colitis (UC) and Crohn disease are classified as inflammatory bowel disease (IBD). While Crohn disease can affect any part of the GI tract (from the oral cavity to the anus), inflammation in UC is generally restricted to the colon and is specifically limited to the mucosa and submucosa. Ulcerative colitis UC is characterized by periods of exacerbation and remission, and, generally, oral lesions coincide with exacerbations of the colonic disease. Lesions in the colon consist of areas of hemorrhage and ulceration, along with abscesses. Cutaneous involvement consists of similar ulcerations that may arise on the buttocks, abdomen, thighs, and face, although in rare cases patients may develop pyoderma vegetans.[1, 2] In the oral cavity, aphthous ulcers or angular stomatitis occurs in as many as 5-10% of patients, although hemorrhagic ulcers can occur.[3] Rarely, patients can develop pyostomatitis vegetans (PSV), the oral counterpart of pyoderma vegetans. Also see Ulcerative Colitis. Crohn disease Crohn disease is an idiopathic inflammatory disorder that can involve the entire GI tract with transmural inflammation and noncaseating granulomas. The prevalence of Crohn disease varies significantly between populations. In North America, the incidence of Crohn disease can be as high as 20.2 cases per 100,000 population. [4] Although formerly considered a disease of Western nations, the incidence is rising in Asia. Similarly, it has long been observed that the incidence of Crohn disease is higher at northern latitudes than at southern latitudes.[4] There is a well- documental bimodal age distribution associated with the onset of Crohn disease: the peak incidence occurs in the second and third decades of life, with a second, smaller peak in the sixth and seventh decades.[4] Genetics have also been implicated in the development of the disease since certain populations (ie, Ashkenazi Jewish populations) have a much higher risk for the development of the disease; more recently, Crohn disease‒associated genetic loci have been identified.[4] Changes to the gut microbiome and various environmental risk factors (including smoking, hygiene, and dietary practices) have also been implicated in the disease’s onset or progression.[4] Symptoms of Crohn disease include intermittent attacks of diarrhea, constipation, abdominal pain, and fever.[5] Patients may develop malabsorption and subsequent malnutrition.[5] Systemic features of Crohn disease include arthritis, clubbing of the fingers, and sacroiliitis.[5] Skin findings include knifelike fissures and ulcerations, as well as fistulae. Vulvar manifestations, such as fissures, edema, tenderness to palpation, and nonspecific aphthae, have also been reported.[5] Cutaneous manifestations of Crohn disease may also be noncontiguous: metastatic Crohn disease is defined as a granulomatous inflammation of the skin that is not contiguous with the GI tract. Although well described, metastatic Crohn disease may present a diagnostic challenge since its clinical presentation is quite variable and may occur without a history of GI disease.[5] Nonspecific, reactive skin findings in patients with Crohn disease include erythema nodosum, pyoderma gangrenosum, and Sweet syndrome.[6] http://emedicine.medscape.com/article/1081029-overview Pagina 1 di 36 Oral Manifestations of Systemic Diseases: Overview, Gastrointestinal Diseases, Nutritional Disease 12/09/16, 11:27 Intraoral involvement occurs in up to 50% of patients with Crohn disease and may precede intestinal involvement.[7] Oral manifestations can prove crucial in diagnosis and usually parallel the intestinal disease course.[8] The severity of oral lesions may indicate the severity of the systemic disease and, as such, may be used as a marker for intestinal impairment.[9] However, oral lesions identified following the diagnosis and control of Crohn disease may not always reflect recurring intestinal disease, but rather treatment adverse effects.[7, 10] Specific oral findings of Crohn disease include diffuse lip, gingival, or mucosal swelling; cobblestoning of the buccal mucosa and gingiva; mucosal tags; and fissures at the angles of the mouth.[11, 12] These lesions are considered specific, because histologic examination reveals evidence of noncaseating granulomatous change.[2, 11, 12] For example, lip fissures may initially resemble angular cheilitis, but often is identified by histology as granular cheilitis. Oral granulomas may occur in isolation, without GI involvement. However, the term orofacial granulomatosis is not specific to Crohn disease, but rather encompasses a variety of other disorders, including sarcoidosis, Melkersson-Rosenthal syndrome, and tuberculosis.[13] Thus, it is not surprising that some patients with orofacial granulomatoses may subsequently develop intestinal manifestations of Crohn disease, but histologic similarities between the oral lesions and the intestinal lesions are evident. Nonspecific oral findings of Crohn disease (ie, lesions for which histology does not reveal granulomatous change) are more common than the specific oral findings.[12] They include PSV, which is discussed in a separate section below, and aphthous ulcers. Approximately 5% of patients with Crohn disease have aphthous ulcers, which are indistinguishable from classic aphthae.[2] Of note, patients may also develop dental caries.[14] Patient-reported symptoms may include significant pain, impaired speech, or difficulty eating secondary to gingival and mucosal swelling, ulcers, or fissures. Patients who present with oral findings suggestive of Crohn disease warrant a full systemic evaluation, including referral for colonoscopy and biopsy with histopathologic correlation. The most common histologic findings in both oral and intestinal Crohn disease are noncaseating granulomas and both acute and chronic inflammation with lymphocytic and giant cell perivascular infiltrates. Also see Crohn Disease. Pyostomatitis vegetans Although relatively rare, most PSV cases occur concurrently with either Crohn disease or UC.[15] As such, PSV is considered a specific finding of IBD, and its presence can precede GI symptoms by months or years.[6] PSV presents as pustules that result in a “snail track” appearance, erosions, and vegetations involving the labial mucosa of the upper and lower lips, buccal mucosa, and gingival mucosa. While the tongue and floor of the mouth are generally spared, the filiform and fungiform lingual papillae may be atrophic.[2, 12, 16] PSV also affects mucosal membranes in the vagina, nasal passages, and periocular region.[12] Symptoms such as pain and burning may be severe or negligible.[2] Histologic examination reveals intraepithelial and subepithelial eosinophilic miliary abscesses.[2] Gastroesophageal reflux Although gastroesophageal reflux is physiologic and occurs in healthy individuals, gastroesophageal reflux disease (GERD) is defined as recurrent regurgitation of acidic gastric contents (pH 1-2) that causes damage to the esophageal mucosa and structures within the oral cavity. GERD has a prevalence of 18-28% in the United States.[17] The etiology of GERD varies among individuals, but it may be due to slackening of the lower esophageal sphincter, increased intra-abdominal pressure, or delayed gastric emptying. Obesity has been shown to significantly increase one’s risk of developing GERD.[18] Patients with GERD commonly report heartburn, chronic cough, sore throat, globus (lump in the throat), dysphagia, and odynophagia.[19] If untreated, GERD may lead to erosive esophagitis, Barrett esophagus, and esophageal adenocarcinoma.[18] There are no cutaneous manifestations of GERD. Regurgitation of gastric contents is not only caustic to the esophageal mucosa, but because the pH of the oral cavity may fall below 5.5, it has been associated with nonspecific burning oral sensation, mucosal erosions and ulcerations, halitosis, and both xerostomia and hypersalivation.[19, 20] In terms of dentition, chronic exposure to acidic fluids in the oral cavity results in dissolution of the tooth surfaces (dental erosion). This is in contrast to labial enamel erosion seen with exposure to sucking on lemons or in patients with bulimia who are often in the habit of brushing their teeth after purging, which results in a labial or facial loss of enamel. Enamel erosion due to gastric fluid exposure is most commonly seen
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