A 61-year-old female patient presented with a 20-year history of swollen masses in her oral mucosa, which sometimes ulcerated. Clinical examination revealed firm nontender infiltrating masses involving the right lateral side of the tongue (3 x 2 cm) [Figure 1a] and right retromolar trigone (2 x 2 cm) [Figure 1b] with granular surfaces, swelling of the lower lip mucosa with cobblestoning [Figure 1c], and multiple palpable, nontender, firm, and mobile lymph nodes on both sides of the neck. She had no symptoms of fever, fatigue, weight loss, or gastrointestinal disturbance, and she had not undergone any investigation or treatment for her complaints before, except using occasional topical corticosteroids and antiseptic mouthwashes. She denied the role of diet on the lesions. Her medical history revealed diabetes mellitus and family history was positive for hypertension in her mother.
Laboratory data including complete blood cell count, serum biochemistry analysis, urinalysis, and serum C reactive protein, calcium, vitamin B12, folate, and angiotensin converting enzyme values were normal except elevated serum fasting glucose (143 mg/dl). The erythrocyte sedimentation rate was 41 mm/h, and chest radiography was normal with no signs of sarcoidosis or tuberculosis. The Mantoux test and pathergy test were also negative.
An incisional biopsy of the lesion involving the retromolar trigone was performed, and the biopsy materal was split into two pieces for histopathologic examination and mycobacteriologic culture. Soft tissue ultrasonography of the neck revealed lymphadenitis, and the biopsy of the lymph nodes showed noncaseating granulomatous lymphadenitis. The histopathologic examination of the oral lesion together with Erlich-Ziehl-Neelsen stain for acidoresistant bacilli (ARB) and periodic acid schiff (PAS) stain showed well-demarcated granuloma formation consisting of epitheloid cells surrounded by lymphocytes in an edematous stroma [Figure 2]. Although the patient had no gastrointestinal symptoms, a colonoscopy was performed. Minor ulcerations were seen in the descending colon. Mucosal biopsies from the observed lesions revealed aphtous ulcerations; inflammatory bowel disease, neoplasia, and tuberculosis were ruled out. Mycobacterial culture of the oral biopsy sample showed no growth.
Based on history, clinical findings, histopathologic examinations, and laboratory data, sarcoidosis, tuberculosis, systemic fungal infections, and Crohn's disease were excluded, and the patient was finally diagnosed as idiopathic orofacial granulomatosis.
Oral prednisolone 50 mg/day was started for ten days and then tapered 10 mg every week. The treatment was well tolerated by the patient except for slight increases in serum glucose levels, which were regulated by adjusting the insulin dosages. Lesions showed marked improvement, and no recurrence was observed in a 2-year follow-up.