PERSISTENT LIP AND FACIAL SWELLING IN A 9 YEAR OLD BOY

Introduction Orofacial Granulomatosis (OFG) is a chronic granulomatous condition characterized by persistent or recurring swelling of soft tissues in the face. It is thought to be caused by a delayed hypersensitivity reaction involving a Th1-mediated immune response. There have been reports of cases associated with Sarcoidosis and Crohn's disease. Case Description A 9-year old male presented to clinic with 15 months of lip and facial swelling. Episodes last 2-3 weeks with resolution periods of 1-2 weeks. Antihistamines failed to improve the swelling. He has normal growth and development. He denies any fevers, headaches, eye redness/pain, oral/nasal ulcerations, difficulty swallowing, decrease in physical conditioning, nausea/vomiting/diarrhea, or bone/joint/muscle complaints. He was started on high dose steroids which improved his lip swelling but as the prednisone was tapered, the swelling returned. He had a mild elevation in Ig G4 and CRP, positive histone antibodies, and elevated C3/C4 and Ig G levels. His Hereditary Angioedema, Sarcoidosis, and Crohn's disease evaluation were negative. Eventually a biopsy of the vestibule of the anterior mandible was done which showed non-necrotizing granulomas. Discussion OFG symptoms can be indistinguishable to those caused by angioedema, contact dermatitis, melkersson-rosenthal syndrome, and lymphangioedema. OFG is made from a diagnosis of exclusion as biopsy cannot distinguish between OFG and Crohn's or Sarcoidosis. Treatment of this condition can be challenging and include steroids, NSAIDs, antibiotics, and TNF-alfa inhibitors. The goal of this case is to provide insight on this uncommon condition whose symptoms present as known sequela of frequent diseases that allergists encounter in their practice.