Dent 356-11 Miscellaneous Disorders of Oral Mucosa

1. Dent 356-11Miscellaneous Disorders of Oral Mucosa Lingual Tonsil, Foliate Papillitis Geographic Tongue Orofacial Granulomatosis Crohn�s Disease Sarcoidosis Pyostomatitis Vegetans Wegener�s Granulomatosis Progressive Systemic Sclerosis Verruciform Xanthoma Oral Submucous Fibrosis Amyloidosis Oral Pigmentation Age Changes

2. Lingual Tonsil, Foliate Papillitis Lingual tonsillar tissue is located on posterior part of lateral aspect of tongue, with overlying vertical folds known as foliate papillae. Inflammation or reactive hyperplasia of this tissue causes enlargement known as foliate papillitis. Obstruction of tonsillar crypts may cause cyst formation resulting in oral lymphoepithelial cyst. Occasional accessory oral tonsils are found elsewhere in the oral cavity.

3. Geographic Tongue(Benign Migratory Glossitis) Common condition of unknown etiology, affecting ~1% of population. Irregular, partially depapillated, red areas on anterior 2/3rds of dorsal tongue. Margins often outlined by a thin white line. Frequently associated with fissured tongue. Appears as small, gradually enlarging lesion(s), which heal and reappear in another location (migratory). Usually asymptomatic, sometimes sensitive to acidic & spicy foods. Similar lesions occasionally affect other parts of oral mucosa (migratory or geographic stomatitis or erythema migrans).

4. Geographic Tongue(Benign Migratory Glossitis) Debatable association with psoriasis in view of histopathologic similarity. Histopathologic features: - acanthosis at edges. - dense neutrophil infiltration of epithelium (microabscesses) & lamina propria, with underlying chronic inflammation.

5. Orofacial Granulomatosis A clinical term used to describe common clinicopathological manifestations of a variety of disorders. Clinically: recurrent or persistent diffuse enlargement of lips and cheeks or as diffuse facial swelling. Histopathologically: non-caseating chronic granulomatous inflammation with or without giant cells, and edema of tissue.

6. Orofacial Granulomatosis Possible causes: Crohn�s disease Sarcoidosis Infective granulomas Foreign bodies Melkersson-Rosenthal syndrome Allergies Idiopathic.

7. Crohn�s Disease A chronic granulomatous disease involving any part of GIT, most commonly terminal ileum. Oral lesions may occur in established intestinal disease, or may be the presenting feature. Chronic course with periods of quiescence interrupted by episodes of varying severity & duration. Unknown etiology, possibly reaction to external agent, may be infective. Abdominal pain & nutritional deficiencies due to malabsorption.

8. Crohn�s Disease Oral manifestations: Diffuse swellings of lips & cheeks (orofacial granulomatosis). Edematous hyperplastic buccolabial mucosa with fissuring (cobblestone appearance). Edematous hyperplastic enlargements of buccolabial mucosa involving sulci, with polypoid tag-like lesions or deep folds. Aphthous or linear fissure-like ulcers. Atrophic glossitis due to vitamin B12 malabsorption.

9. Sarcoidosis Systemic chronic granulomatous disorder of unknown etiology. Suggested unusual response to exogenous agent. Young adults. Bilateral hilar lymphadenopathy, pulmonary infiltration, skin or eye lesions. Insidious onset, signs & symptoms disappear in time but sometimes leave residual swelling.

10. Sarcoidosis Oral mucosal & GI involvement rare. May present as submucosal, painless red nodules and as erythema, granularity, or hyperplasia of gingiva. May present as orofacial granulomatosis. Salivary gland involvement, usually parotid. Minor salivary gland biopsy is sometimes a helpful diagnostic tool. Heerfordt syndrome (uveoparotid fever): - uveitis - parotitis - facial paralysis.

11. Sarcoidosis Histopathologic Features: Small non-caseating granulomas consisting of macrophages & epithelioid histiocytes, often with Langhans-type giant cells. Laboratory Findings: Raised ESR, hyperproteinemia, elavated ACE, & hypercalciuria.

12. Pyostomatitis Vegetans Rare disorder associated with inflammatory bowel disease, especially UC. Lips & cheeks diffusely inflamed with erosions, fissured ulcers, pustules and vegetations. Cervical lymphadenitis & pyrexia. Skin lesions (pyodermatitis) may occur at the same time. Severity of oral lesions varies with that of bowel involvement. UC also predisposes to recurrent aphthous stomatitis.

13. Wegener�s Granulomatosis Necrotizing, destructive granulomatous inflammation of respiratory tract, and generalized necrotizing vasculitis. Rhinitis, sinusitis, cough, & hemoptysis. Autoantibodies against cytoplasmic components of neutrophils and monocytes (c-ANCA). May be controlled with corticosteroids & cytotoxic medications, otherwise fatal due to renal failure.

14. Wegener�s Granulomatosis Oral involvement common and may be the presenting feature in the form of: - characteristic hyperplastic gingivitis (strawberry gingivitis). - heavily inflamed granular exophytic lesions. - deep necrotic oral ulceration.

15. Progressive Systemic Sclerosis(Scleroderma) Chronic multi-system disease characterized by diffuse fibrosis of skin and internal organs. Facial involvement results in restricted mouth opening & expressionless (mask-like) face. Females aged 20-50 years. Generalized widening of PDL space on oral radiographs. Association with other autoimmune diseases : LE, RA, Sj�gren�s syndrome suggests autoimmune etiology.

16. Verruciform Xanthoma Uncommon lesion mostly occurring on gingiva and hard palate. Flat or slightly raised lesion with papillary surface. Probably occurs in reaction to release of lipid from degenerating epithelial cells. Histopathologic Features: - hyperplastic epithelium with papillary projections. - CT papillae infiltrated by lipid-containing foam cells (xanthoma cells).

17. Oral Submucous Fibrosis Insidious chronic disease which affects any part of oral mucosa and may extend to pharynx and esophagus. Progressive stiffening of oral mucosa associated with underlying fibrosis. Exclusively limited to Asiatic Indians. Strongly linked to areca nut chewing habits (betel quid, betel nut) with possible genetic susceptibility. Regarded as a premalignant condition often associated with epithelial atrophy and dysplasia.

18. Amyloidosis Extracellular deposition of fibrillar proteinaceous material-amyloid- in various tissues. AL type: fragments of immunoglobulin light chains in primary (idiopathic) amyloidosis and amyloidosis associated with multiple myeloma. AA type: amyloid A protein in secondary or reactive amyloidosis which complicates chronic inflammatory lesions and some malignancies.

19. Amyloidosis Oral manifestations described with primary amyloidosis and MM: - Macroglossia. - Petechiae, ecchynoses, hemorrhagic bullae, ulcers. - Localized amyloid deposits in the form of yellowish macules or papules. - Widespread symptomless infiltration of oral tissues. Involvement of salivary glands may result in xerostomia. -

20. Amyloidosis Diagnosis is confirmed by biopsy (Congo red stain). Even without apparent oral involvement, biopsy of tongue or gingiva may be a useful diagnostic test. Diagnosis for generalized amyloidosis is poor and death results from organ failure.

21. Oral Pigmentation:I. Exogenous Pigmentation Superficial staining of mucosa. Black hairy tongue. Foreign bodies: amalgam tattoo, miscellaneous. Heavy metal salts.

22. Oral Pigmentation:II. Endogenous Melanin Pigmentation Developmental causes: racial pigmentation, pigmented nevi, Peutz-Jeghers syndrome. Acquired causes: - associated with systemic disease: Addison�s disease, pulmonary disease, HIV infection. - associated with hyperkeratosis, chronic inflammation, and lichen planus. - smoker�s melanosis.* Acquired causes: - drug-induced. - idiopathic oral melanotic macule (oral freckle). - lentigo simplex. Neoplastic causes: melanoma in situ and melanoma.

23. Oral Pigmentation: III. Other endogenous pigments  blood breakdown products and disturbances of iron metabolism.

24. Age Changes in the Oral Mucosa Atrophy of lingual mucosa. CT becomes more fibrosed, less vascular, & less cellular. Atherosclerotic changes in arteries. Sublingual varices. Increased prominence of Fordyce�s granules. Foliate papillitis. Effects of systemic disease, nutritional deficiences, & side-effects of medications are more frequent in the elderly.