title: Orofacial granulomatosis as early manifestation of Crohn's disease: report of a case in a paediatric patient.
abstract: BACKGROUND
  Orofacial granulomatosis (OFG) is a controversial entity mainly characterised by recurrent or permanent soft tissue swelling of sudden onset in the orofacial area with a histologic appearance of granulomatous inflammation. Differential diagnosis includes local diseases and systemic conditions, such as Crohn's disease (CD). A case of OFG in a paediatric patient is reported here, focusing on the clinical features, diagnostic procedures, treatment and long-term outcome.
  
  
  METHODS
  A 7 year-old boy presented with persistent and prominent lip swelling and painful oral mucosa lesions of six months duration. A biopsy of the lower labial mucosa revealed granulomatous inflammation consistent with OFG. The oral manifestations were managed with topical and intralesional corticosteroids. His medical history included gastrointestinal disturbances, perianal skin folds and bloody stools that raised the suspicion of CD. Colonoscopy showed inflammation without clear evidence of CD. One year later, repeated bowel investigation provided evidence suggestive of CD and the patient was placed under systemic treatment. Two years after the initial diagnosis the patient is free of oral or other pathological findings.
  
  
  CONCLUSIONS
  The differential diagnosis in cases of orofacial swellings with histological granulomatous inflammation includes a variety of local and systemic diseases, diagnosis and management of which require full investigation and cooperation by a team of healthcare providers.
authors.affiliation: 1st Department of Pediatrics, Medical School, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: A
authors.initials: A
authors.lastname: Sarra
authors.affiliation: Department of Oral Pathology and Medicine, Dental School, National and Kapodistrian University of Athens, Greece.
authors.forename: N G
authors.initials: NG
authors.lastname: Nikitakis
authors.affiliation: Department of Oral Pathology and Medicine, Dental School, National and Kapodistrian University of Athens, Greece.
authors.forename: A
authors.initials: A
authors.lastname: Daskalopoulos
authors.affiliation: 1st Department of Pediatrics, Medical School, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: G
authors.initials: G
authors.lastname: Chouliaras
authors.affiliation: Department of Oral Pathology and Medicine, Dental School, National and Kapodistrian University of Athens, Greece.
authors.forename: A
authors.initials: A
authors.lastname: Sklavounou-Andrikopoulou
authors.affiliation: 1st Department of Pediatrics, Medical School, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: K
authors.initials: K
authors.lastname: Athanasaki
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D003424:Crohn Disease / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D051261:Granulomatosis, Orofacial / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male
chemical_list: D000305:Adrenal Cortex Hormones; D005765:Gastrointestinal Agents