Department of Dermatology, Medical School, University of São Paulo, São Paulo, Brazil,
Department of General Pathology, Dental School, University of São Paulo, São Paulo, Brazil.
Dermatology. 2021;237(4):635-640. doi: 10.1159/000513446. Epub 2021 Feb 12.
To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD).
This review was conducted according to PRISMA guidelines and a search of the PubMed, MEDLINE, and Embase databases, and the Cochrane Library in March 2020, using keywords and MeSH terms associated with "orofacial granulomatosis," "Crohn disease," and their variants, with no language restrictions and across all age groups. All relevant articles were accessed in full text. Single case reports and articles on sarcoidosis, allergy, ulcerative colitis, and infectious diseases were excluded from the analysis.
We retrieved 507 reports on OFG. The mean age at onset was 23.3 years (range 2-89 years). A total of 240 (47.3%) females and 267 (52.6%) males were included. CD was present in 93 children aged <16 years (68.3%) and in 43 adults (31.9%). In most cases, the OFG appeared before the CD. The most common clinical manifestations were intraoral mucosa abnormalities (n = 251; 49.5%), lower-lip swelling (n = 249; 49.1%), upper-lip swelling (n = 227; 44.7%), and gingivae (n = 193; 38.7%). Patients with concurrent CD were more likely to experience involvement of the buccal sulcus. Key Messages: OFG presents primarily as a solo entity. The OFG that was associated with CD was present in 93 children aged under 16 years (68.3%) and in 43 adults (31.9%). Childhood onset of OFG carries with it a higher risk of developing CD.
系统回顾所有口腔颌面部肉芽肿（OFG）病例，并评估 OFG 与克罗恩病（CD）之间的关联。
本综述按照 PRISMA 指南进行，于 2020 年 3 月检索了 PubMed、MEDLINE 和 Embase 数据库以及 Cochrane 图书馆，使用了与“口腔颌面部肉芽肿”、“克罗恩病”及其变体相关的关键词和 MeSH 术语，无语言限制且涵盖所有年龄段。全文获取所有相关文章。单病例报告和关于结节病、过敏、溃疡性结肠炎和传染病的文章被排除在分析之外。
我们检索到 507 篇关于 OFG 的报告。发病时的平均年龄为 23.3 岁（范围 2-89 岁）。共纳入 240 名（47.3%）女性和 267 名（52.6%）男性。93 名年龄<16 岁的儿童（68.3%）和 43 名成人（31.9%）患有 CD。在大多数情况下，OFG 先于 CD 出现。最常见的临床表现是口腔黏膜异常（n=251；49.5%）、下唇肿胀（n=249；49.1%）、上唇肿胀（n=227；44.7%）和牙龈肿胀（n=193；38.7%）。患有并发 CD 的患者更有可能出现颊沟受累。
OFG 主要表现为单一实体。与 CD 相关的 OFG 见于 93 名年龄<16 岁的儿童（68.3%）和 43 名成人（31.9%）。OFG 的儿童发病与发生 CD 的风险较高相关。
