Crohns Disease

Generalised fibrous hyperplasia/hypertrophy of the gingiva can be familial or drug-induced.

Hereditary gingival fibromatosis is a rare condition that is usually inherited as an autosomal dominant trait [20]. It can affect all of the gingiva, often in a symmetrical manner. It may be associated with hypertrichosis, coarsening of the facial features and neurological problems such as epilepsy and mental retardation. The condition usually first affects adolescents, but occasionally it can involve the deciduous dentition. The enlargement is usually most conspicuous in the interdental areas and affects the palatal and lingual gingiva as well as the labial and buccal aspects. The overgrowths may be so florid that the teeth involved are almost completely buried (pseudo-anodontia). The overgrowths are rounded, smooth, firm and pale-coloured. Treatment is by surgical removal of the redundant fibrous tissue (gingivecto-my), but the condition often recurs.

Drug-associated gingival hypertrophy is seen in about half of patients using the anti-epileptic drug phenyto-in for long-term treatment [1]. Other drugs producing a similar reaction include cyclosporin (~30% of patients)

Crohn's disease is a multisystem disorder characterised microscopically by non-caseating, epithelioid granulomas. Despite extensive investigations the cause remains unknown. Recently, genetic predisposition has been extensively investigated and among others, a susceptibility gene has been identified on chromosome 16q [18]. Smoking also appears to play a critical role in some patients [60]. Although an infective aetiology, particularly mycobacterial, has been long suspected, critical proof is lacking [173]. The most likely candidate organism is the Mycobacterium avium subspecies paratuberculosis [68]. Granulomatous vasculitis, possibly initiated by the measles virus, has been proposed as a significant factor in the pathogenesis [178]. However, blood vessel involvement may be a secondary phenomenon rather than a primary event [120]. Patients are usually children and young adults and there is a male preponderance. Associations between alterations in the intestinal microflora are also suspected and a statistically significant association between Crohn's disease and previous antibiotic use has been proposed [29]. The condition may be made worse by cigarette smoking [80].

Oral lesions are relatively common in patients with Crohn's disease of the lower gastro-intestinal tract [57, 185], but may be the presenting symptoms. They include swelling of the lips and cheeks (Fig. 3.17), recurrent aphthae, painful, indolent linear ulcers in the vestibular sul-ci, cobblestone thickening of the buccal mucosa, mucosal tags and hyperplastic, granular gingivitis [138]. The palate, tongue and pharynx, including the palatine ton

Fig. 3.17. Crohn's disease showing unilateral lip swelling with fis- Fig. 3.18. Crohn's disease showing a small, irregular epithelioid suring and peri-oral dermatitis granuloma and patchy chronic inflammation

sil, are only rarely involved [19]. Extraorally, there may be angular stomatitis and vertical fissuring of the lips, and perioral erythema and scaling.

Oral lesions may precede, or accompany, bowel symptoms, but in a significant number of cases intestinal disease is subclinical. In patients with active bowel disease there may be atrophic glossitis secondary to malabsorption of the haematinics iron, vitamin B12 or folate.

Microscopy of oral lesions typically shows oedema of the superficial corium with lymphangiecta-sia and diffuse and focal aggregates of small lymphocytes. Non-caseating epithelioid granulomas with or without multinucleated giant cells are present in about 90% of cases [138]. However, granulomas may be small and poorly formed and may only be present in the underlying muscle so that they can be easily missed, especially if the biopsy is superficial (Fig. 3.18). Granulomas can also sometimes be seen in the minor salivary glands. Aggregates of mononuclear cells or granulomas may be seen bulging into or within the lumina of lymphatics. This feature has been termed endovasal granulomatous lymphangiitis [128]. Dilated lymphatics, with or without associated granulomas, are characteristic of Crohn's disease elsewhere in the alimentary tract [120].

As many as 80-90% of patients with orofacial lesions that resemble those of Crohn's disease, both clinically and microscopically, have no gastro-intestinal signs or symptoms and do not develop gut disease [159]. The term orofacial granulomatosis (OFG) has been introduced to describe this group of patients [184]. OFG, therefore, is a diagnosis based on the exclusion of other causes of granulomatous inflammation, particularly sarcoidosis, tuberculosis and other mycobacterial infections, and Crohn's disease itself. Up to 60% of patients with OFG are atopic [86] and some patients appear to show an idiosyncratic intolerance to a variety of foods or additives, including cinnamonaldehyde, carvone, carnosine, sun yellow, benzoates and monosodium glutamate [169], and to metallic compounds containing cobalt [146]. In many cases there is a partial or complete resolution of symptoms following withdrawal of the provoking agent.

OFG may be part of a spectrum of diseases that includes Melkersson Rosenthal syndrome (MRS) and cheilitis granulomatosa (Miescher's syndrome). MRS, in its complete form, is a triad of fissured tongue, labial or facial swelling due to granulomatous inflammation, and facial nerve palsy, which may be the first indication of the disease [192]. Cheilitis granulomatosa is probably merely an isolated manifestation of OFG.

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