[Melkersson-Rosenthal syndrome]

Melkersson-Rosenthal syndrome is a rare entity and is, in its complete presentation, characterized by the association of recurrent orofacial swelling, peripheral facial palsy and plicated tongue. Clinical diagnosis of incomplete forms, frequently described, is based on major and minor features, currently accepted by numerous clinicians. For most investigators, Miescher's granulomatous macrocheilitis is a monosymptomatic form of the affection. Histopathological findings reveal non-caseating epithelioid cell granulomas, with sometimes perivascular pathognomnic arrangement, which typically shows an obliterant epithelioid lymphangitis. The etiology of the disorder is unknown; a possible association with Crohn's disease has been proposed. Local or general corticosteroids therapy is usually proposed possibly with a cheiloplastic procedure.