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Oral Manifestations of Systemic Disease Heidi L Oral manifestations of systemic disease Heidi L. Gaddey, MD On examination, the oral cavity may exhibit manifesta- he oral cavity can lend insight into underlying health in tions of underlying systemic disease and serve as an both adults and children. In 2000, the US Surgeon General 1 indicator of overall health. Systemic diseases with oral highlighted the links between oral and general health. findings include autoimmune, hematologic, endocrine, T Many systemic diseases first present as, or can be identified based and neoplastic processes. Autoimmune disease may on, changes within the oral cavity. This article will review select manifest as oral ulcerations, changes in the salivary and common oral cavity findings in adult and pediatric patients and parotid glands, and changes in the tongue. Patients with the systemic diseases with which they may be associated. hematologic illnesses may present with gingival bleeding or tongue changes such as glossitis, depending on the Autoimmune diseases etiology. Oral changes associated with endocrine illness Lupus erythematosus are variable and depend on the underlying condition. Systemic lupus erythematosus (SLE) and discoid lupus ery- Neoplastic changes include metastatic lesions to the thematosus (DLE) present with oral findings in 8%-45% and 2 bony and soft tissues of the oral cavity. Patients with 4%-25% of patients, respectively. SLE is the most common chronic diseases such as gastroesophageal reflux and vascular collagen disorder in the United States. Associated oral eating disorders may present with dental erosions that lesions can vary greatly in appearance, manifesting as ulcer- 3,4 cause oral pain or halitosis. In the pediatric population, ations, erythema, or hyperkeratosis (Fig 1). Cheilitis may also 3 oral changes can be related to rare cancers, such as be present. Oral lesions associated with DLE are typically ulcer- Langerhans cell histiocytosis, or infectious etiologies, ated, atrophic, and erythematous; they usually demonstrate a 3 such as Kawasaki disease. In both adults and pediatric central zone with radiating, fine, white striae. These oral lesions patients, poor oral health has been linked to poorer are identical to erosive lichen planus; however, the absence of health outcomes overall. Thorough history taking and skin findings in patients with erosive lichen planus typically 4 physical examination by dentists may aid in determining excludes the diagnosis of DLE. the underlying etiology of oral changes and allow for Lesions can be treated with topical corticosteroids, systemic earlier intervention by medical colleagues. antimalarial drugs, or systemic immunosuppressive agents, if needed, based on severity. Received: July 18, 2017 Revised: September 7, 2017 Systemic sclerosis (scleroderma) Accepted: September 12, 2017 Systemic sclerosis is characterized by dense collagen deposition within the tissues and ranges from localized to systemic disease. 5 Key words: autoimmune, endocrine, hematologic, Females are more commonly affected. Skin findings range from oral manifestations Raynaud phenomenon to masklike and “mouse” facies. Oral findings are variable, including changes to the lips and mouth (Fig 2). The lips appear pursed, and opening of the mouth may 6 be limited. Xerostomia is common; the tongue appears smooth, 5 as do the palatal rugae. On panoramic radiographs, mandibular 6,7 resorption may be noted. Treatment is focused on limiting further progression, al- though often the changes are irreversible. Range of motion exercises may be beneficial to aid in mouth opening, and oral 6 hygiene instruction should be provided to the patient. Sjögren syndrome Published with permission of the Academy of General Dentistry. Sjögren syndrome (SS) is characterized by xerostomia and © Copyright 2017 by the Academy of General Dentistry. xerophthalmia and more commonly affects females. It can be All rights reserved. For printed and electronic reprints of this article classified as primary or secondary, the latter if SS is associated for distribution, please contact [email protected]. with another autoimmune illness. Associated autoimmune 8 conditions include rheumatoid arthritis, SLE, and scleroderma. Oral manifestations of SS include parotid enlargement and A collaboration between General Dentistry findings related to decreased saliva, such as increased risk of and American Family Physician dental caries, infections, and dysphagia. Saliva is often thick Special ORAL-SYSTEMIC HEALTH Section 23 Oral manifestations of systemic disease A B Fig 1. Oral ulcer in a patient with Fig 2. A. Pursed lips and masklike facies of a patient with scleroderma. B. Bone resorption (arrows) systemic lupus erythematosus. related to scleroderma. (Reprinted from Albilia et al with permission from the Canadian Dental (Reprinted from Islam NM, Association.6) Bhattacharyya I, Cohen DM. Common oral manifestations of systemic disease. Otolaryngol Clin North Am. 2011;44(1): 161-182, with permission from Elsevier.4) Fig 3. Fissured tongue related to Sjögren Fig 4. Amyloid deposition of the tongue in Fig 5. Ulcerations associated with syndrome. (Reproduced with permission a patient with amyloidosis. (Reprinted from pemphigus vulgaris. (Reproduced with from Common tongue conditions in Mangold AR, Torgerson RR, Rogers RS 3rd. permission from Oral manifestations primary care. American Family Physician. Diseases of the tongue. Clin Dermatol. of systemic disease. American Family 2010;81(5):627-634.9 Copyright ©2010 2016;34(4):458-469, with permission from Physician. 2010;82(11):1381-1388.14 American Academy of Family Physicians. Elsevier.11) Copyright ©2010 American Academy of All rights reserved.) Family Physicians. All rights reserved.) 4 or absent, and the oral mucosa may be dry, red, and wrinkled. Miscellaneous The tongue may be atrophic or fissured with deep grooves and Other autoimmune illnesses associated with oral findings 4,9 malodorous due to food trapping (Fig 3). Sjögren syndrome is include pemphigus vulgaris, Wegener granulomatosis, Crohn complicated by non-Hodgkin lymphoma, primarily low-grade disease, Behçet syndrome, benign mucus membrane pemphi- mucosal-associated lymphoid tissue lymphomas, with a goid, sarcoidosis, and lichen planus. Painful oral ulcerations are 10 reported incidence of 3.5%-11%. common among all of these conditions, which may be difficult 14 Treatment includes the use of sialogogues, systemic mus- to distinguish from one another (Fig 5). A positive Nikolsky carinic agonists, and orosalivary gland stimulants as well as sign—dislodgment of the superficial epidermis with a shearing instruction in oral hygiene to protect the dentition. force—is consistent with pemphigus. Wegener granulomatosis 4 often presents as “strawberry gingivitis.” Crohn disease is Amyloidosis associated with diffuse swelling, localized mucogingivitis, and Amyloidosis can be classified as 2 types: organ-limited or sys- cobblestoned mucosa. Nodules, tissue tags, polyps, and pyo- temic. The oral soft tissues are rarely affected in organ-limited stomatitis vegetans can also occur in association with Crohn 15 amyloidosis. Systemic amyloidosis can lead to macroglossia due disease (Fig 6). 11 to amyloid deposition in the tongue (Fig 4). Tongue lesions present as nodular or diffuse enlargement with subsequent ulcer- Hematologic diseases 12,13 ations or hemorrhages. Biopsy can confirm the diagnosis. Blood disorders can present differently within the oral cavity Treatment depends on the type of amyloidosis and the source based on the underlying condition. Mucosal pallor and atrophy of amyloid production. are common in anemia, whereas hemorrhages and gingival 24 GENERAL DENTISTRY November/December 2017 Fig 6. Pyostomatitis vegetans in a patient Fig 7. Atrophic glossitis. Fig 8. Magenta tongue in a patient with with Crohn disease. (Reprinted from (Reproduced with permission pernicious anemia. (Reprinted from Islam Atarbashi-Moghadam et al with permission from Oral manifestations NM, Bhattacharyya I, Cohen DM. Common from JCDR Research and Publications Ltd.15) of systemic disease. oral manifestations of systemic disease. American Family Physician. Otolaryngol Clin North Am. 2011;44(1): 2010;82(11):1381-1388.14 161-182, with permission from Elsevier.4) Copyright ©2010 American Academy of Family Physicians. All rights reserved.) Fig 9. Diffuse, hemorrhagic enlargement of the Fig 10. Idiopathic purpura and hemorrhagic gingiva in a patient with leukemia. (Reproduced with bullae resulting from thrombocytopenia. permission from Oral manifestations of systemic (Reproduced with permission from Oral disease. American Family Physician. 2010;82(11):1381- manifestations of systemic disease. American 1388.14 Copyright ©2010 American Academy of Family Family Physician. 2010;82(11):1381-1388.14 Physicians. All rights reserved.) Copyright ©2010 American Academy of Family Physicians. All rights reserved.) bleeding are often associated with hematopoietic neoplasms Leukemia or coagulopathies. Treatment of oral manifestations of hema- Gingival enlargement, petechiae, mucosal bleeding, and ulcer- tologic illnesses is focused on management of the underly- ations are findings within the oral cavity that are suggestive of 14 ing disease in coordination with the patient’s hematologist leukemia (Fig 9). Hemorrhages can occur on both the hard or oncologist. and soft palates once the platelet counts fall below 20,000/μL 4 (20 × 109/L). Mucosal ulcers may also be present. Bony infiltra- Anemia tion by malignant cells can lead to ulcerations
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