Oral Manifestations of Systemic Diseases

Synonyms and related keywords: gastrointestinal diseases, Crohn disease, Crohn's disease, ulcerative colitis, gastroesophageal reflux disease, GERD, gastroesophageal reflux, reflux disease, hepatitis, chronic liver disease, hepatitis C, hematologic disorders, anemia, Langerhans cell histiocytosis, histiocytosis X, Letterer-Siwe disease, Hand-Sch�ller-Christian disease, Hand-Schuller-Christian disease, connective tissue disorders, Sjögren syndrome, Sjögren's syndrome, Sjogren syndrome, Kawasaki disease, Kawasaki's disease, mucocutaneous lymph node syndrome, pulmonary disorders, Wegener's granulomatosis, Wegener granulomatosis, sarcoidosis, multisystem disease, amyloidosis, drug-induced conditions, inhaled steroids, gingival hyperplasia, AIDS, acquired immunodeficiency syndrome, candidiasis, herpes simplex virus, HSV, hairy leukoplakia, HL, Kaposi's sarcoma, Kaposi sarcoma, cytomegalovirus, CMV, cutaneous diseases, psoriasis, acanthosis nigrans, AN.

Author: Jeffrey M Casiglia, DMD, DMSc, Assistant Professor, Indiana University School of Dentistry; Faculty, Department of Oral Medicine, Infection and Immunity, Harvard School of Dental Medicine

Coauthor(s): Ginat W Mirowski, DMD, MD, Adjunt Associate Professor, Departments of Oral Pathology, Medicine, and Radiology, Indiana University School Medicine

Jeffrey M Casiglia, DMD, DMSc, is a member of the following medical societies: American Academy of Oral Medicine, and American Dental Association

Editor(s): Franklin Flowers, MD, Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, University of Florida College of Medicine; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; and William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System.

The oral cavity is the portal of entry to the GI tract. Lined by stratified squamous epithelium, the tissues of the mouth are often involved when individuals have conditions affecting the GI system. These may be immune-mediated or chemically mediated processes.

Crohn disease

Crohn disease is an idiopathic disorder that can involve the entire GI tract with transmural inflammation, noncaseating granulomas, and fissures. This disease is most common in Western countries and is slightly more prevalent among white males. The peak incidence is in the second and third decades of life, with a second peak occurring in the sixth and seventh decades. Symptoms of Crohn disease include intermittent attacks of diarrhea, constipation, abdominal pain, and fever. Patients may develop malabsorption and subsequent malnutrition. Fissures or fistulas may occur in persons with chronic disease.

Intraoral involvement in Crohn disease occurs in 8-9% of patients and may precede intestinal involvement. With oral involvement, the likelihood of extraintestinal manifestations is greater. Extraintestinal features are also common in persons with Crohn disease, and these may manifest systemically as arthritis, clubbing of the fingers, sacroiliitis, and erythema nodosum.

Orofacial symptoms of Crohn disease include (1) diffuse labial, gingival, or mucosal swelling; (2) cobblestoning of the buccal mucosa and gingiva; (3) aphthous ulcers; (4) mucosal tags; and (5) angular cheilitis. Noncaseating granulomas are characteristic of orofacial Crohn disease. Oral granulomas may occur without characteristic alimentary involvement (orofacial granulomatoses). However, the term orofacial granulomatoses encompasses a variety of other disorders, including sarcoidosis, Melkersson-Rosenthal syndrome, and, rarely, tuberculosis. Whether patients with orofacial granulomatoses will subsequently develop intestinal manifestations of Crohn disease is uncertain, but histologic similarities between the oral lesions and the intestinal lesions are obvious.

Labial swelling is most often a cosmetic complaint, but it can be a painful manifestation of the disease. Gingival and mucosal involvement may cause difficulty while eating. The pattern of swelling, inflammation, ulcers, and fissures is similar to that of the lesions occurring in the intestinal tract. Acute and chronic inflammation, with lymphocytic and giant cell perivascular infiltrates, and lymphoid follicles are the most common histologic findings in oral and GI Crohn disease. Noncaseating granulomas are present in biopsy samples in a number of cases. Increased dental caries and nutritional deficiencies may be related to decreased saliva production and malabsorption in the intestinal tract.

Oral findings as described above warrant a full systemic evaluation for intestinal Crohn disease, including referral for colonoscopy and biopsy with histopathologic correlation. Oral involvement may precede systemic manifestations and symptoms. Negative findings on GI evaluations should be repeated in patients with oral symptoms. The severity of oral lesions may coincide with the severity of the systemic disease, and it may be used as a marker for intestinal impairment (Halme, 1993).