Department of Interdisciplinary Medicine, Aldo Moro University Bari, Italy.
Department of Emergency and Organ Transplantation, Aldo Moro University Bari, Italy.
Eur J Paediatr Dent. 2020 Mar;21(1):66-69. doi: 10.23804/ejpd.2020.21.01.13.
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.
口面肉芽肿病是一个描述性术语,通常包括具有相似临床和显微镜特征的多种疾病。它通常用于描述口腔和颌面区域软组织的持续增大。
我们报告了 8 例儿科克罗恩病患者(年龄 9 至 13 岁)的口腔病变的主要临床特征,这些患者的口腔病变是首发临床表现。
口面肉芽肿病的临床表现变化多样,但通常以反复发作的面部肿胀为特征,主要累及嘴唇,伴有或不伴有口腔内表现,是发病时最常见的单一临床征象。该疾病与结节病和克罗恩病等系统性疾病的关联在文献中已有广泛报道。在儿科年龄,口面肉芽肿病可能经常代表克罗恩病的肠外表现,口腔病变可能是未知肠道疾病的首发征象。在儿科患者中,由于口面肉芽肿病可能在克罗恩病之前数年发生,且常常是唯一明显的疾病活跃病灶,因此诊断具有挑战性。
对于肠道克罗恩病的早期诊断,必须检测到特定的口腔表现,这些表现通常先于无痛性牙龈增大(弥漫性唇和颊黏膜肿胀、口腔鹅卵石样改变、颊沟溃疡和黏膜小结节)和/或非特异性或辅助性表现(唇炎、鳞屑性口周红斑疹和明显的口腔脓肿形成、唇裂和舌裂、舌炎和口疮性口炎)。