Intralymphatic granulomas as a pathogenic factor in cheilitis granulomatosa/Melkersson-Rosenthal syndrome: report of a case with immunohistochemical and molecular studies.

Orofacial granulomatosis, an uncommon immunologically mediated disorder, includes cheilitis granulomatosa and Melkersson-Rosenthal syndrome. It is clinically characterized by recurrent or persistent swelling of the orofacial tissues with a spectrum of other orofacial features and sometimes with neurological symptoms. The pathological findings are varied but are often characterized by the presence of noncaseating granuloma. We present a new case of orofacial granulomatosis with unusual histopathological findings, namely, intralymphatic granulomas. These may be the cause of the tissue edema. We demonstrated, by immunohistochemical studies, the lymphatic nature of the vessels affected by the granulomatous process.

Therapeutic response to thalidomide in Melkersson-Rosenthal syndrome: a case report.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology characterized by a triad of symptoms: recurrent orofacial swelling, relapsing facial palsy. and a fissured tongue. A differential diagnosis must be made with other granulomatous diseases, such as sarcoidosis and oral Crohn disease; however, the histologic findings of noncaseating, sarcoidal granulomas support the diagnosis of MRS. RESULTS: Many therapeutic modalities have been described for this disease. In this case report, we present a patient with MRS that was treated with thalidomide because of the identification of tumor necrosis factor a in the lesion by immunohistochemical analysis. This is the first reported detection of tumor necrosis factor a in an MRS lesion, as well the first reported use of thalidomide to treat this clinical condition.