Melkersson-Rosenthal syndrome

Melkersson-Rosenthal syndrome is a rare entity and is, in its complete pres entation, characterized by the association of recurrent orofacial swelling, peripheral facial palsy and plicated tongue. Clinical diagnosis of incompl ete forms, frequently described, is based on major and minor features, curr ently accepted by numerous clinicians. For most investigators, Miescher's g ranulomatous macrocheilitis is a monosymptomatic form of the affection. His topathological findings reveal non-caseating epithelioid cell granulomas, w ith sometimes perivascular pathognomnic arrangement, which typically shows an obliterant epithelioid lymphangitis. The etiology of the disorder is unk nown; a possible association with Crohn's disease has been proposed, Local or general corticosteroids therapy is usually proposed possibly with a chei loplastic procedure.