Sign Up for a Free Account
  • Updated 10.21.2020
  • Released 04.20.2004
  • Expires For CME 10.21.2023

Melkersson-Rosenthal syndrome

Introduction

Overview

Recurrent facial palsy with orofacial swelling and fissuring of the tongue is known as 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 (1883-1930), a Swedish physician, described a 35-year-old woman with facial edema and paralysis (45). Shortly after, in 1931, Curt Rosenthal (1892-1937), 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 (56).

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 (25; 60). 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).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, 
including video clips of 
neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of 
neurology in 1,200 
comprehensive articles.

Questions or Comment?

MedLink, LLC

10393 San Diego Mission Rd, Suite 120

San Diego, CA 92108-2134

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com