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  • Updated 05.14.2024
  • Released 04.20.2004
  • Expires For CME 05.14.2027

Melkersson-Rosenthal syndrome

Introduction

Overview

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

Key points

• Melkersson-Rosenthal syndrome should be suspected in cases of recurrent facial palsy associated with swelling of orofacial structures 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 (1883-1930), a Swedish physician, described a 35-year-old woman with facial edema and paralysis (56). Shortly after, in 1931, Curt Rosenthal (1892-1937), a German neurologist, described three 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 (69).

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 (34; 74). 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 (95).

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