Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management

Melkersson-Rosenthal Syndrome (MRS) is a rare neuro-mucocutaneous disorder characterised by a triad of recurrent facial palsy, orofacial swelling and fissured tongue. The complete triad occurs in fewer than 25% of cases, complicating timely diagnosis and management. The present review focuses on the...

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Main Authors: Megha Tidke, Pramod Borghare, Piyush Pardhekar, Raza Salim Sheikh
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2025-06-01
Series:Journal of Clinical and Diagnostic Research
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Online Access:https://jcdr.net/articles/PDF/21058/76474_CE[Ra1]_F(IS)_QC(SD_SS)_PF1(RI_SL)_redo_PFA(IS)_PB(Pr_OM)_PN(IS).pdf
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author Megha Tidke
Pramod Borghare
Piyush Pardhekar
Raza Salim Sheikh
author_facet Megha Tidke
Pramod Borghare
Piyush Pardhekar
Raza Salim Sheikh
author_sort Megha Tidke
collection DOAJ
description Melkersson-Rosenthal Syndrome (MRS) is a rare neuro-mucocutaneous disorder characterised by a triad of recurrent facial palsy, orofacial swelling and fissured tongue. The complete triad occurs in fewer than 25% of cases, complicating timely diagnosis and management. The present review focuses on the clinical features, diagnostic difficulties and management approaches of MRS, hence establishing its clinical relevance and treatment results. A literature survey is conducted based on case reports, clinical trials and MRS reviews. The approach towards the diagnosis and different modes of treatments with multidisciplinary management analysis, is done. MRS is a condition with some diagnostic difficulties because it often presents variably and even mimics other diseases like angioedema, Bell’s palsy and Crohn’s disease. The gold standard of histopathology is the presence of non caseating granulomas. The acute symptoms can be treated with corticosteroids, as early as 30 to 60 mg/day; however, long-term treatment has to be avoided because of complications such as osteoporosis. Immunocompressants, like methotrexate and azathioprine, are used in refractory cases. Some of the considerations and measures would be surgical interventions, cheiloplasty and nerve decompression. However, these are risky to be accompanied by scarring, nerve damage, recurrence of symptoms and so on. A multidisciplinary approach involving dermatologists, neurologists and psychologists is critical for improving patient outcomes and quality of life. In conclusion, MRS remains a diagnostic and therapeutic challenge because of its persistence and various presentations. Accurate and early diagnosis, immediate intervention and a multidisciplinary approach to treatment will be necessary to prevent complications. Future studies on the genetic and immunological basis of MRS will help develop better diagnostic and therapeutic options.
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spelling doaj-art-2cd5cf2f455c464fa8af829cb2b90eed2025-08-20T02:34:35ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2025-06-01196ME01ME0510.7860/JCDR/2025/76474.21058Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and ManagementMegha Tidke0Pramod Borghare1Piyush Pardhekar2Raza Salim Sheikh3Assistant Professor, Department of Shalakya Tantra, Mahatma Gandhi Ayurved College, Hospital and Research Centre, Wardha, Maharashtra, India.Associate Professor, Department of Shalakya Tantra, Mahatma Gandhi Ayurved College, Hospital and Research Centre, Wardha, Maharashtra, India.Assistant Professor, Department of Shalakya Tantra, Mahatma Gandhi Ayurved College, Hospital and Research Centre, Wardha, Maharashtra, India.Intern, Department of Shalya Tantra, Mahatma Gandhi Ayurved College, Hospital and Research Centre, Sausar, Madhya Pradesh, India.Melkersson-Rosenthal Syndrome (MRS) is a rare neuro-mucocutaneous disorder characterised by a triad of recurrent facial palsy, orofacial swelling and fissured tongue. The complete triad occurs in fewer than 25% of cases, complicating timely diagnosis and management. The present review focuses on the clinical features, diagnostic difficulties and management approaches of MRS, hence establishing its clinical relevance and treatment results. A literature survey is conducted based on case reports, clinical trials and MRS reviews. The approach towards the diagnosis and different modes of treatments with multidisciplinary management analysis, is done. MRS is a condition with some diagnostic difficulties because it often presents variably and even mimics other diseases like angioedema, Bell’s palsy and Crohn’s disease. The gold standard of histopathology is the presence of non caseating granulomas. The acute symptoms can be treated with corticosteroids, as early as 30 to 60 mg/day; however, long-term treatment has to be avoided because of complications such as osteoporosis. Immunocompressants, like methotrexate and azathioprine, are used in refractory cases. Some of the considerations and measures would be surgical interventions, cheiloplasty and nerve decompression. However, these are risky to be accompanied by scarring, nerve damage, recurrence of symptoms and so on. A multidisciplinary approach involving dermatologists, neurologists and psychologists is critical for improving patient outcomes and quality of life. In conclusion, MRS remains a diagnostic and therapeutic challenge because of its persistence and various presentations. Accurate and early diagnosis, immediate intervention and a multidisciplinary approach to treatment will be necessary to prevent complications. Future studies on the genetic and immunological basis of MRS will help develop better diagnostic and therapeutic options.https://jcdr.net/articles/PDF/21058/76474_CE[Ra1]_F(IS)_QC(SD_SS)_PF1(RI_SL)_redo_PFA(IS)_PB(Pr_OM)_PN(IS).pdfangioedemacheilitis granulomatosacorticosteroidsfacial palsyimmunosuppressantsorofacial oedemapathogenesis
spellingShingle Megha Tidke
Pramod Borghare
Piyush Pardhekar
Raza Salim Sheikh
Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
Journal of Clinical and Diagnostic Research
angioedema
cheilitis granulomatosa
corticosteroids
facial palsy
immunosuppressants
orofacial oedema
pathogenesis
title Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
title_full Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
title_fullStr Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
title_full_unstemmed Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
title_short Melkersson-Rosenthal Syndrome: A Review of Clinical Presentation, Diagnosis and Management
title_sort melkersson rosenthal syndrome a review of clinical presentation diagnosis and management
topic angioedema
cheilitis granulomatosa
corticosteroids
facial palsy
immunosuppressants
orofacial oedema
pathogenesis
url https://jcdr.net/articles/PDF/21058/76474_CE[Ra1]_F(IS)_QC(SD_SS)_PF1(RI_SL)_redo_PFA(IS)_PB(Pr_OM)_PN(IS).pdf
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AT razasalimsheikh melkerssonrosenthalsyndromeareviewofclinicalpresentationdiagnosisandmanagement