Melkersson-Rosenthal syndrome

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released April 20, 2004; last updated June 17, 2016; expires June 17, 2019

This article includes discussion of Melkersson-Rosenthal syndrome, Melkersson syndrome, Rossolimo-Melkersson-Rosenthal syndrome, Melkersson-Rosenthal-Scheuermann syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Recurrent facial palsy with orofacial swelling and fissuring of the tongue is known as the Melkersson-Rosenthal syndrome. This chapter provides a review of the clinical features, hypothesized pathophysiology, and current management of this syndrome.

Key points

 

• Melkersson-Rosenthal syndrome should be suspected in cases of recurrent facial palsy associated with swelling of orofacial structures and/or a furrowed tongue.

 

• Biopsy of suggestive lesions typically reveals noncaseating granulomas with perivascular and lymphatic inflammatory cell infiltration.

 

• The pathophysiology involves an immunologically mediated inflammatory response.

 

• Treatment consists of various immune-suppressing and modulating agents, along with surgical correction of disfiguring lesions as necessary.

Historical note and terminology

In 1928, Ernst Melkersson, a Swedish physician, described a 35-year-old woman with facial edema and paralysis. Shortly after, in 1931, Curt Rosenthal, a German neurologist, described 3 patients who had fissured or plicated tongues in addition to orofacial swelling and facial palsies, and he also proposed a link between the triad of symptoms. Subsequently, the triad of clinical findings came to be known as the Melkersson-Rosenthal syndrome (Rosenthal 1931).

When the characteristic facial swelling is confined to the lips and occurs in a monosymptomatic or oligosymptomatic form (ie, in isolation or in conjunction with either a furrowed tongue or facial palsy), it is referred to as Miescher cheilitis or cheilitis granulomatosa (Greene and Rogers 1989; Sciubba and Said-Al-Naief 2003). If granulomatous swelling is localized mainly to the eyelids, the disorder is referred to as granulomatous blepharitis and considered a monosymptomatic form of the Melkersson-Rosenthal syndrome (Yeatts and White 1997).

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