Oral lesions as first clinical manifestations of Crohn's disease in paediatric patients: a report on 8 cases.

Abstract

Oro-facial granulomatosis is a descriptive term commonly encompassing a variety of conditions that exhibit similar clinical and microscopic features. It is generally used to describe persistent enlargement of the soft tissues of the oral and maxillofacial region. We report on the salient clinical features of 8 cases of Crohn's disease in paediatric patients (age range from 9 to 13 years old), with oral lesions as first clinical manifestations. The clinical presentation of oro-facial granulomatosis is highly variable but usually recurrent facial swelling, mainly in the lips with or without intraoral manifestations, is the single most common clinical sign at onset. The association with systemic conditions such as sarcoidosis and Crohn's disease has been widely reported in literature. In paediatric age, oro-facial granulomatosis may frequently represent an extra-intestinal manifestation of Crohn's disease and oral lesions can be the first sign of an unknown intestinal disease. The diagnosis in paediatric patients is challenging as oro-facial granulomatosis may precede Crohn's disease by several years, frequently remaining the only evident active focus of the disease. The detection of specific oral manifestations often preceded by painless gingival enlargement (diffuse lip and buccal mucosal swelling, oral cobblestoning, buccal sulcus ulceration and mucosal tags) and/or unspecific or ancillary ones (cheilitis, scaly perioral erythematous rashes and frank intraoral abscess formation, labial and tongue fissuring, glossitis and aphthous stomatitis) is mandatory for the early diagnosis of intestinal Crohn's disease.