Orofacial granulomatosis presenting as persistent lip swelling: review of 6 new cases

Orofacial granulomatosis (OFG) is an uncommon disease, which presents usually as a persistent swelling of the soft tissues in the orofacial region and is characterized histologically by a granulomatous inflammation. The term orofacial granulomatosis is used in the literature to describe a nonspecific granulomatous inflammation. A subset, cheilitis granulomatosa (CG), which presents clinically as persistent lip swelling, is a granulomatous inflammation of unknown origin of the lips. CG may also be part of the triad of the Melkersson-Rosenthal syndrome (MRS) and some consider it as an oligosymptomatic form of MRS.

In a retrospective study, we reviewed the clinical records of all patients between the years 1990 and 2002 with the histologic diagnosis of granulomatous inflammation of the lip from the Department of Pathology at the Montreal General Hospital. The age at biopsy, sex, site of lesion, other signs of MRS, signs and symptoms of systemic granulomatous disease, and the treatment were compiled.

Six subjects were included in the study, 5 females and 1 male. Four had swelling of the lower lip and 2 of the upper lip. In one patient a history of facial nerve palsy was reported and in another one a mild fissured tongue was present. No signs or symptoms of systemic granulomatous disease were noted. The treatment consisted of intralesional steroids with good results in 5 out of 6 patients.

A thorough work-up to eliminate other etiologies of granulomatous disease is essential when a patient presents with granulomatous inflammation of the lip. CG seems to respond well to steroid treatment and the need for surgery is minimal and should be reserved for recalcitrant cases.