Clinical features and long-term behaviour of orofacial granulomatosis

Background: There remain few detailed reports of the clinical features and long term response to therapy of substantial groups of patients with orofacial granulomatosis (OFG).

Objectives: The aim of this study was to determine the clinical, haematological and histopathological features of a large cohort of patients with OFG and to define the long-term response to therapy.

Patients/methods: Clinically relevant haematological, serological and histopathological data of 49 patients with OFG who attended a single Oral Medicine unit in the UK were retrospectively examined. The analyzed parameters included early and late clinical features, adverse side effects of therapy, and long-term disease behaviour.

Results: The observation period of this cohort varied from 1 to 22 years (median 2.0). Recurrent oro-facial swelling was the most common (55.1%) abnormality at disease onset, while intra-oral ulcers as the only presenting sign were observed in 28.6% of patients . 70% later developed a variety of different intra-oral and/or extra-oral manifestations of OFG. Topical corticosteroids/immunosuppressants alone led to disease control/stabilization in about one-third of patients although the majority of patients required systemic and/or intra-lesional corticosteroids or other immunosuppressants to reduce disease. The clinical behaviour was typically remitting, there being frequent oscillations between partial and complete improvement. Adverse effects of therapy were rare. 12.2% of the group, all on systemic agents, had adverse drug reactions such as nausea, vomiting, diarrhoea, cutaneous rash and sensory neuropathy. Haematological and serological abnormalities were infrequent and not of clinical significance.

Conclusions: Orofacial granulomatosis has a variable clinical presentation and response to therapy. Spontaneous remission is uncommon, but treatment is generally safe.