The diagnosis of Melkersson-Rosenthal syndrome (MRS) is rarely established, mainly because abortive cases are common. The principal symptoms are recurrent circumscribed swelling mostly of the orofacial region, peripheral facial paralysis and lingua plicata, which is nowadays no longer regarded as pathognostic. The syndrome is defined best as general disorder respectively "recurrent edematous granulomatosis" with specific pathoclisis to "angioneurotic intermittent edemas". The--non obligatory--classical histological finding is cheilitis granulomatosa. Etiopathogenesis, clinic, symptomatology and therapy are dealt with. Disorders of the function of the salivary glands, vegetative marginal symptoms, extrafacial intermittent edemas, cranial nerve syndromes and mostly mild peripheral neural and/or CNS syndromes along with psychiatric disorders are communicated. Atypical constellations of symptoms, differential diagnosis and neuropathological results are discussed. Finally neurovegetative, pathophysiological and phylogenetic aspects as well as therapeutic principles are mentioned.
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