Saalman Robert, Mattsson Ulf, Jontell Mats
Department of Pediatrics, The Queen Silvia Children's Hospital, The Sahlgrenska Academy, Göteborg University, Göteborg, Sweden.
Acta Paediatr. 2009 Jul;98(7):1162-7. doi: 10.1111/j.1651-2227.2009.01295.x. Epub 2009 Apr 17.
Orofacial granulomatosis (OFG) is a rare clinical entity with orofacial swelling in association with oral lesions such as mucosal oedema, ulcerations and mucosal tags. The aim of this prospective study was to evaluate the connection between OFG in childhood and systemic disease.
During a 3-year period, eight children (9-16 years old) who had been referred to the clinic of oral medicine were diagnosed solely with OFG. Thus, none of them had any known systemic disease or gastrointestinal symptoms at the time of referral. The children were then medically examined and followed up for 6-8 years at the department of paediatrics for systemic disease with specific emphasis on inflammatory disorders elsewhere in the gastrointestinal tract.
During follow-up, four patients were diagnosed with Crohn's disease (CD). Further, one girl was found to have a food allergy-induced OFG, with delayed-type hypersensitivity to oats. One boy developed both diabetes and celiac disease during the follow-up. Only two patients had no diagnosis of systemic disease at the end of the observation period.
OFG in childhood seems to be frequently related to systemic disease, and children with OFG should be referred to a paediatrician for examination and follow-up.
口面部肉芽肿病(OFG)是一种罕见的临床病症,表现为口面部肿胀并伴有口腔病变,如黏膜水肿、溃疡和黏膜赘生物。这项前瞻性研究的目的是评估儿童OFG与全身性疾病之间的关联。
在3年期间,8名转诊至口腔医学诊所的儿童(9至16岁)被确诊仅患有OFG。因此,他们在转诊时均无已知的全身性疾病或胃肠道症状。随后对这些儿童进行医学检查,并在儿科进行了6至8年的随访,重点关注胃肠道其他部位的炎症性疾病。
在随访期间,4名患者被诊断为克罗恩病(CD)。此外,一名女孩被发现患有食物过敏诱发的OFG,对燕麦存在迟发型超敏反应。一名男孩在随访期间患了糖尿病和乳糜泻。在观察期结束时,只有两名患者未被诊断出患有全身性疾病。
儿童OFG似乎常与全身性疾病相关,患有OFG的儿童应转诊至儿科医生处进行检查和随访。