Yared Zenebe Zewde1. 1. Department of Neurology, School of Medicine, College of Health Sciences, Addis Ababa University, P.O. Box 41690, Addis Ababa, Ethiopia. yaredzene121@gmail.com.
Abstract
BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder of unknown cause, clinically characterized by a triad of recurrent facial palsy, orofacial swelling, and fissured tongue. It is frequently seen in females in their second and third decades of life. MRS is diagnosed based on clinical features and it is rarely possible to observe all the classical triad symptoms at the same time. The disorder may cause recurring peripheral facial palsy that is wrongly diagnosed as recurrent Bell's palsy CASE PRESENTATION: A 25-year-old female patient was presented to the neurology clinic of Tikur Anbessa Specialized Hospital in Addis Ababa complaining of recurrent left-side peripheral facial weakness, facial swelling and fissured tongue of 5 days duration. Her past medical history was positive for similar symptoms, for which she was diagnosed with Bell's palsy and received oral corticosteroid treatment. On examination left side lower facial swelling with flat naso-labial fold and fissured tongue were identified. After excluding other mimickers, she was diagnosed with Melkersson-Rosenthal syndrome and completely recovered with high dose of corticosteroid treatment. CONCLUSION: Melkersson-Rosenthal syndrome may present with the classic triads of symptoms, but mostly it shows an incomplete clinical pattern. Therefore, when clinicians including allergists encountered patients with facial swelling and facial palsy, they should have to consider MRS in their differential diagnosis and specifically assess for recurrent facial palsy and fissured tongue. Unlike true angioedema, the facial swelling in MRS often develops gradually and it might cause permanent swelling with cosmetic disfigurement from multiple relapses, which can be prevented by early detection and timely initiation of treatment.
BACKGROUND:Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder of unknown cause, clinically characterized by a triad of recurrent facial palsy, orofacial swelling, and fissured tongue. It is frequently seen in females in their second and third decades of life. MRS is diagnosed based on clinical features and it is rarely possible to observe all the classical triad symptoms at the same time. The disorder may cause recurring peripheral facial palsy that is wrongly diagnosed as recurrent Bell's palsy CASE PRESENTATION: A 25-year-old female patient was presented to the neurology clinic of Tikur Anbessa Specialized Hospital in Addis Ababa complaining of recurrent left-side peripheral facial weakness, facial swelling and fissured tongue of 5 days duration. Her past medical history was positive for similar symptoms, for which she was diagnosed with Bell's palsy and received oral corticosteroid treatment. On examination left side lower facial swelling with flat naso-labial fold and fissured tongue were identified. After excluding other mimickers, she was diagnosed with Melkersson-Rosenthal syndrome and completely recovered with high dose of corticosteroid treatment. CONCLUSION:Melkersson-Rosenthal syndrome may present with the classic triads of symptoms, but mostly it shows an incomplete clinical pattern. Therefore, when clinicians including allergists encountered patients with facial swelling and facial palsy, they should have to consider MRS in their differential diagnosis and specifically assess for recurrent facial palsy and fissured tongue. Unlike true angioedema, the facial swelling in MRS often develops gradually and it might cause permanent swelling with cosmetic disfigurement from multiple relapses, which can be prevented by early detection and timely initiation of treatment.