Orofacial granulomatosis – a 20‐year review

Abstract

Orofacial granulomatosis (OFG) is the presence of persistent enlargement of the soft tissues of the oral and maxillofacial region, characterized by non-caseating granulomatous inflammation in the absence of diagnosable systemic Crohn's disease (CD) or sarcoidosis. Over 20 years have passed since OFG was first described and an extensive review of the literature reveals that there is no consensus whether OFG is a distinct clinical disorder or an initial presentation of CD or sarcoidosis. Furthermore, the precise cause of OFG is still unknown although several theories have been suggested including infection, genetic predisposition and allergy. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. Regular clinical review is indicated to identify the development of gastrointestinal or systemic involvement. The aim of this review was to analyse the developments in our understanding of the aetiology, pathogenesis and treatment protocols, with particular emphasis on management and outcomes of OFG since this entity was first described in 1985.

Orofacial Granulomatosis Associated with Crohn's Disease

“…OFG is a rare granulomatous disorder, characterized by persistent enlargement of the soft tissues of the oral and maxillofacial region 3 . Recurrent facial swelling, with/without intraoral manifestations, was the single most common presentation at onset 4 .…”

“…The nomenclature of OFG lacks specificity 3 . Recently, a question has been posed to determine whether OFG is a manifestation of a separate and specific inflammatory bowel disease 5 .…”

Ano-genital Granulomatosis and Crohn’s Disease: A Case Series of Males Presenting with Genital Lymphoedema

“…21 OFG is a rare clinical entity manifest by lymphoedema of the face and oral cavity and shares many of the histological characteristics of AGG. 22 OFG is also reported to overlap with intestinal CD in 20-40% of cases. 18,23 Notably in our series, four patients had OFG, 2 of whom had a triad of AGG,OFG and CD.…”

Orofacial granulomatosis: insights from an immunohistochemical evaluation

“…When associated with fissured tongue and facial nerve palsy, it is named Melkersson-Rosental syndrome [3] . Moreover, OFG may represent the earliest manifestation of Crohn's disease, a granulomatous condition that can affect any part of the gastrointestinal tract [4,5,[7][8][9] .…”