Clinical characteristics and treatment of Melkersson-Rosenthal syndrome - overview of six patients

Rašković, Sanvila; Bolpacic, Jasna; Perić-Popadić, Aleksandra; Jovičić, Žikica; Mišković, Rada; M, Bogić

doi:10.2298/mpns1512401r

Cited by 4 publications

(4 citation statements)

References 8 publications

(12 reference statements)

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“…Other alternatives used are dietary changes, avoiding possible allergenics and nutraceuticals supplements [ 25 ]. Other medications such as anti-inflammatories, steroids, and antihistamines have been utilized to improve symptoms [ 26 ]. The effectivity of the pharmacological therapies is moderate and relapses occur frequently.…”

Section: Discussionmentioning

confidence: 99%

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Quintero

Camargo-Camargo

López

et al. 2021

Case Reports in Neurological Medicine

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Introduction. Melkersson–Rosenthal syndrome (MRS) is a neuromucocutaneous disorder characterized by the following classic symptom triad: peripheral facial paralysis, orofacial edema, and scrotal or fissured tongue. It is rare, and since most of the patients are oligo- or monosymptomatic, it makes it difficult to diagnose. Clinical Case. We present a 26-year-old male patient with a history of sickle cell trait, untreated snoring, and left peripheral facial paralysis when he was 11 years old. This was an overall 20-day clinical profile that started with left peripheral facial paralysis, which was accompanied by moderate-intensity occipital pulsatile headaches. Additionally, the patient experienced paresthesias in the tongue and feelings of labial edema. After one week, he manifested peripheral facial paralysis on the right side. Physical examination revealed bilateral peripheral facial paralysis, mild labial edema, and a scrotal or fissured tongue. The patient received corticosteroids, which resulted in improvement of the edema and facial paralysis. Discussion. MRS is a rare disorder that predominantly affects women, typically starting in their 20s or 30s. The etiology is unknown. However, a multifactorial origin that involves environmental factors and a genetic predisposition has been proposed, which causes a dysfunction of the local immune system and autonomic nervous system (ANS) and an appearance of granulomatous inflammation in the lips and tongue. Facial paralysis usually appears later on; however, it can occur from its clinical debut. There are no curative treatments. Therapy is focused on modulating the patient's immune response, and relapses are frequent.

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Section: Discussionmentioning

confidence: 99%

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Quintero

Camargo-Camargo

López

et al. 2021

Case Reports in Neurological Medicine

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“…Though its etiology is not precisely known, it is thought that various infections, delayed susceptibility reactions, stress, and autoimmune and genetic factors may be related to the etiopathogenesis. [1][2][3][4][5][6][7][8] The diagnosis can be made directly based on the presence of the full triad of symptoms; when only 1or 2 are observed, a skin biopsy from the facial edema should be performed and demonstrate granulomatous cholestasis pathologically. [9] Steroids, anti-inflammatory agents, antibiotics, and immunosuppressants may be used in treatment; however, spontaneous recovery also occurs.…”

Section: Introductionmentioning

confidence: 99%

Melkersson–Rosenthal syndrome: Case Report

Aydın¹

2018

South Clin Ist Euras

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Melkersson-Rosenthal Syndrome (MRS) is a rare disease characterized by the triad of recurring facial paralysis, a fissured tongue, and recurring swelling of the lips and/or face. The etiology is unknown; it may be caused by hereditary factors or conditions occurring later in life. Observation of all 3 of the classic manifestations is rare; most often the symptoms are seen alone or in a pair, and they may appear at any stage of life. This report is the description of the case of a 51-year-old woman who was diagnosed with MRS based on her history and clinical features.

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“…Its main clinical presentation includes swelling of the lips, face, and/or gums 3, 4 . Current understanding on the aetiology and pathogenesis of CG are still incomplete 5, 6 .…”

Section: Introductionmentioning

confidence: 99%

“…Moreover, the evaluation of lip swelling and therapeutic efficacy of CG does not follow a standard criteria or achieve consensus. The evaluation of therapeutic effects across most studies has been mainly based on the patients’ self-perception and doctors’ subjective evaluation 9–11 . Objective criteria for evaluation of treatment are still lacking.…”

Section: Introductionmentioning

confidence: 99%

Development of a facescan 3D facial reconstruction technology method for quantitative evaluation of cheilitis granulomatosa

Wang

Liu

et al. 2017

Sci Rep

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We explored the applicability of Facescan three-dimensional (3D) facial reconstruction technology for adjunctive diagnosis and therapeutic evaluation of cheilitis granulomatosa (CG) in 33 patients with CG and 29 healthy controls at the Dept. of Oral Medicine, Peking University, School and Hospital of Stomatology (PKUSS), from January 2015 to May 2016. The Facescan structured-light 3D facial reconstruction scanner was used to scan the scope of lips in both groups, in order to acquire 3D morphological data of the lips. The lengths of six characteristic line segments were measured from the 3D lip model of the two groups, and the acquired data were compared. The results showed that the distance between the labiale superius and labiale inferius, and the lengths of the upper and lower vermilion borders showed significant differences between the CG and control groups, by using the 3D lip model. Thus, Facescan 3D facial reconstruction technology showed good reproducibility in the evaluation of lip swelling in CG patients, and it can be used to analyse the degree of lip swelling and evaluate the therapeutic efficacy of different treatments for CG.

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Clinical characteristics and treatment of Melkersson-Rosenthal syndrome - overview of six patients

Cited by 4 publications

References 8 publications

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Melkersson–Rosenthal syndrome: Case Report

Development of a facescan 3D facial reconstruction technology method for quantitative evaluation of cheilitis granulomatosa

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Clinical characteristics and treatment of Melkersson-Rosenthal syndrome - overview of six patients

Cited by 4 publications

References 8 publications

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Presentation of Bilateral Facial Paralysis in Melkersson–Rosenthal Syndrome

Melkersson–Rosenthal syndrome: Case Report

﻿Development ﻿﻿of﻿ ﻿﻿a facescan 3D facial reconstruction technology method for quantitative evaluation of cheilitis granulomatosa

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Development of a facescan 3D facial reconstruction technology method for quantitative evaluation of cheilitis granulomatosa