Orofacial Granulomatosis and Crohn Disease: Coincidence or Pattern? A Systematic Review

Author(s)

Gavioli, Camila Fatima Biancardi ^[1] ; Florezi, Giovanna Piacenza ^[2] ; Dabronzo, Maria Luiza Ducati ^[1] ; Jimenez, Marcela Ricaurte ^[1] ; Nico, Marcello Menta Simonsen ^[1] ; Lourenco, Silvia Vanessa ^[2]

Affiliation

^[1] Univ Sao Paulo, Sch Med, Dept Dermatol, 255 Doutor Eneas Carvalho Aguiar Ave, BR-05403000 Sao Paulo - Brazil

^[2] Univ Sao Paulo, Sch Dent, Dept Gen Pathol, Sao Paulo - Brazil

• Document type: Review article
• Source: DERMATOLOGY; v. 237, n. 4, p. 635-640, JUN 2021.
• Web of Science Citations: 0

Abstract

Background: To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD). Summary: 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. Results: 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. (AU)

• FAPESP's process: 17/26990-8 - Melkersson-Rosenthal syndrome: epidemiological, histopathological and serological profile and the interface with orofacial granulomatosis diagnosis
• Grantee: Marcello Menta Simonsen Nico
• Support Opportunities: Regular Research Grants