Orofacial granulomatosis: clinical features and long-term outcome of therapy.
BACKGROUND
Orofacial granulomatosis (OFG) is a chronic inflammatory disorder characterized by persistent or recurrent soft tissue enlargement, oral ulceration, and a variety of other orofacial features. There remain few detailed reports of the clinical features and long-term response to therapy of substantial groups of patients with OFG.


OBJECTIVE
The aim of this study was to determine retrospectively the clinical, hematologic, and histopathological features of a large case series of patients with OFG. In addition the long-term response to therapy was examined.


METHODS
Clinically relevant data of 49 patients with OFG who attended a single oral medicine unit in the United Kingdom were retrospectively examined. The analyzed parameters included diagnostic features, clinical manifestations, and outcomes and adverse side effects of therapy.


RESULTS
Labial swelling was the most common presenting clinical feature at diagnosis (75.5%), followed by intraoral mucosal features other than ulceration such as cobblestoning and gingival enlargement (73.5%). Mucosal ulceration was observed in 36.7% of patients whereas extraoral facial manifestations such as cutaneous erythema and swelling were present in 40.8% of patients. Of the 45 patients who required treatment, 24 (53.3%) were treated with topical corticosteroids/immunosuppressants only, whereas 21 (46.7%) received a combined therapy (topical plus systemic corticosteroids/immunosuppressants and/or intralesional corticosteroids). The long-term outcome analysis showed complete/partial resolution of tissue swelling and oral ulceration in 78.8% and 70% of patients, respectively.


CONCLUSIONS
The main limitation of the current study was its retrospective design and methodology including differences in reporting clinical features and outcome.


CONCLUSIONS
OFG can show multiple facial and mucosal clinical features. Long-term treatment with topical and/or combined therapy is needed in the majority of patients. Response to therapy is highly variable even though in the long-term complete/partial disease resolution can be obtained in the majority of patients. Mucosal ulceration tends to be more recalcitrant than orofacial swelling. Adverse side effects of therapy are rare.
Oral Medicine, University College London Eastman Dental Institute and University College London Hospital Eastman Dental Hospital, London, UK. K.aljohani@eastman.ucl.ac.uk
Khalid A
KA
Al Johani
David R
DR
Moles
Tim A
TA
Hodgson
Stephen R
SR
Porter
Stefano
S
Fedele
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D051261:Granulomatosis, Orofacial / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult