Orofacial Granulomatosis as the Initial Presentation of Crohn’s Disease in an Adolescent

Abstract

Orofacial granulomatosis (OFG) is a rare and heterogeneous clinical condition that presents with chronic swelling of the oral or facial tissues due to granulomatous inflammation. It is histologically characterized by noncaseating giant cell granulomata and epithelioid histiocytes. OFG includes the previously recognized clinical entities of Melkersson-Rosenthal syndrome and cheilitis granulomatosa (Miescher’s cheilitis). A consistently effective medical treatment is not currently available. We describe an adolescent patient with a history of recurrent orofacial swelling preceding gastrointestinal symptoms by several years. He exhibited clinical and histological changes consistent with the diagnosis of OFG. It was resistant to standard therapies such as topical corticosteroids. Thorough history taking and clinical examination suggested Crohn’s disease (CD), and further tests confirmed the diagnosis. The patient improved promptly after initiating oral treatment for CD with mesalazine and prednisolone. We conclude that OFG may be under- or misdiagnosed since the clinical manifestation may be misleading and its course is independent of or even preceding CD. Thus, patients with OFG should be asked about gastrointestinal symptoms consistent with CD. Those with suspicious symptoms should undergo a careful gastrointestinal evaluation, possibly including enteroclysis and complete gastrointestinal endoscopic examination, especially when the patient’s history is conspicuous.

Orofacial Granulomatosis Associated with Crohn's Disease

“…Recently, a question has been posed to determine whether OFG is a manifestation of a separate and specific inflammatory bowel disease 5 . Other authors also suggested that OFG is a descriptive term and the specific cause of these lesions is unknown 6 . Bogenrieder et al 6 reported that OFG is not an entity but is rather a generic name applied as a provisional diagnosis for orofacial lesions in patients where the associated systemic or local disease process has not yet been identified.…”

“…Other authors also suggested that OFG is a descriptive term and the specific cause of these lesions is unknown 6 . Bogenrieder et al 6 reported that OFG is not an entity but is rather a generic name applied as a provisional diagnosis for orofacial lesions in patients where the associated systemic or local disease process has not yet been identified. OFG may precede gastrointestinal disease by several years and may be the only obvious focus of disease 6,7 .…”

“…Bogenrieder et al 6 reported that OFG is not an entity but is rather a generic name applied as a provisional diagnosis for orofacial lesions in patients where the associated systemic or local disease process has not yet been identified. OFG may precede gastrointestinal disease by several years and may be the only obvious focus of disease 6,7 . In this regard, Scully et al 8 found that 37% of patients, having no intestinal symptoms but with clinical evidence of oral CD, had demonstrable intestinal disease.…”

Asymptomatic Inflammatory Bowel Disease Presenting With Mucocutaneous Findings

“…Many patients with orofacial granulomatosis do eventually develop gastrointestinal disease consistent with Crohn's disease. 20,21 "Cobblestoning" refers to nodular granulomatous swellings that result in a cobblestone appearance of the oral mucosa, particularly on the labial and buccal mucosa. Along with mucosal tags, cobblestoning is highly suggestive of Crohn's disease.…”

Syndrome de Melkersson-Rosenthal. À propos de deux observations

“…Le diagnostic différentiel avec ces deux affections est parfois difficile. [14,15,16] . Dans la sarcoïdose, le granulome est bien diffé-rent de celui du SMR.…”