Melkersson-Rosenthal syndrome and orofacial granulomatosis

The Melkersson-Rosenthal syndrome is a rare disorder of unknown etiology characterized by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. Exacerbations and recurrences are common. The orofacial swelling is characterized by fissured, reddish-brown, swollen, nonpruritic lips or firm edema of the face. The facial palsy is indistinguishable from Bell's palsy. The fissured tongue is seen in one third to one half of patients and, although the least common manifestation, its presence assists in diagnosis. The classic triad is not seen frequently in its complete form; therefore, diagnosis is difficult. This is particularly true because monosymptomatic and oligosymptomatic variants are seen more commonly. Cheilitis granulomatosa of Miescher is an example of a monosymptomatic variant of the Melkersson-Rosenthal syndrome. The histologic findings of noncaseating, sarcoidal granulomas support the diagnosis. These granulomas are not invariably present, and their absence does not exclude the diagnosis of the Melkersson-Rosenthal syndrome. Thus, the Melkersson-Rosenthal syndrome is a disease with elements of orofacial granulomatosis. Orofacial granulomatosis is a clinicopathologic entity describing oral lesions with noncaseating granulomas. The spectrum of this entity includes patients with oral Crohn's disease, patients with oral lesions who will develop typical bowel symptoms of Crohn's disease in the ensuing months to years, patients with tooth-associated infections, patients with sarcoidosis, and patients with food or contact allergies. The value of the clinicopathologic construct of orofacial granulomatosis is to provoke the careful search for provocative causes for the reactive symptom complex of the Melkersson-Rosenthal syndrome.