Granulomatose lymphomatoïde buccale

Abstract

A 57 year old man was referred to us with multiple long-standing, recurring and refractory mouth ulcers involving the palate, the intermaxillary folds, the inner side of the cheeks, the frenum of the tongue, a tonsil, the pharynx, the gums and a lower lip. Multiple biopsies had excluded a lymphoma, a carcinoma and infection. A new biopsy showed a polymorphous granuloma with up to 40% of atypical lymphoid cells. No angiitis was present, but an angiocentric pattern was present in the granuloma. The atypical cells stained positively with OKT3 and OKT4 antisera. The patient also had cardiac rhythm disturbances with defects on the myocardial scan and a proteinuria. Thus, we suspected the diagnosis of lymphomatoid granulomatosis, although there were no pulmonary nodules. Cyclophosphamide and prednisone were given with immediate success. As the doses were gradually decreased, a small relapse occurred. It was controlled by increasing the cyclophosphamide dosage for six months. The patient is now in complete remission more than four years after having discontinued the treatment. Thus, lymphomatoid granulomatosis can be diagnosed on the basis of a biopsy of buccal ulcers and in the absence of the classical pulmonary nodules. We found no evidence that the atypical cells were lymphomatous; rather, the very good response to the treatment points to a curable granulomatosis.