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World J Stomatol. Feb 20, 2015; 4(1): 8-11
Published online Feb 20, 2015. doi: 10.5321/wjs.v4.i1.8
Melkersson-Rosenthal syndrome
Ioannis Chatzistefanou, Petros Zikos, Grigorios Venetis, Konstantinos Vahtsevanos, Konstantinos Antoniades
Ioannis Chatzistefanou, Petros Zikos, Grigorios Venetis, Konstantinos Vahtsevanos, Konstantinos Antoniades, Department of Oral and Maxillofacial Surgery, “G. Papanikolaou” General Hospital, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece
Author contributions: All the authors equally contributed to this paper.
Conflict-of-interest: The authors declare that they have no competing interests.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Ioannis Chatzistefanou, MD, DDS, Department of Oral and Maxillofacial Surgery, “G. Papanikolaou” General Hospital, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece. ioannis.chatzistefanou@gmail.com
Telephone: +30-231-760124 Fax: +30-231-760124
Received: August 12, 2014
Peer-review started: August 14, 2014
First decision: December 18, 2014
Revised: December 22, 2014
Accepted: December 29, 2014
Article in press: December 31, 2014
Published online: February 20, 2015
Processing time: 185 Days and 15.3 Hours
Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare, non-caseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the literature describing the Melkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications met eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS. Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.

Keywords: Melkersson-Rosenthal syndrome; Orofacial swelling; Cheilitis granulomatosa; Facial nerve palsy; Fissured tongue

Core tip: Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of melkersson-Rosenthal syndrome (MRS). The coexistence of orofacial edema with facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS. Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.