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Orofacial granulomatosis: Clinical features and long-term outcome of therapy - 24/04/13

Doi : 10.1016/j.jaad.2009.03.051 
Khalid A. Al Johani, BDS, MSc a, , David R. Moles, PhD, MSc (Hons), MIHPE, DDPH RCS (Eng), FHEA b, Tim A. Hodgson, FDS, FDS (OM) RCS, MRCP (UK), FGDP (UK) a, Stephen R. Porter, PhD, MD, FDSRCS, FDSRCSE a, Stefano Fedele, DDS, PhD a
a Oral Medicine, University College London Eastman Dental Institute and University College London Hospital Eastman Dental Hospital, London, United Kingdom 
b Health Services Research, University College London Eastman Dental Institute and University College London Hospital Eastman Dental Hospital, London, United Kingdom 

Reprint requests: Khalid A. Al-Johani, BDS, MSc, Oral Medicine, UCL Eastman Dental Institute, 256 Gray’s Inn Road, London WC1X 8LD, United Kingdom.

Abstract

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.

Limitations

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.

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Key words : clinical features, long-term outcome, orofacial granulomatosis


Plan


 This work was undertaken at University College London/University College London Hospital Trust who received a proportion of funding from the Department of Health’s National Institute for Health Research Biomedical Research Centre funding scheme. K. Al-Johani received sponsorship from the King Abdulaziz University, Saudi Arabia.
 Conflicts of interest: None declared.


© 2009  American Academy of Dermatology, Inc.. Publié par Elsevier Masson SAS. Tous droits réservés.
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Vol 62 - N° 4

P. 611-620 - avril 2010 Retour au numéro
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