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Melkersson–Rosenthal syndrome (MRS) in children is a rare condition, clinically characterised by a triad of synchronous or metachronous symptoms: recurrent peripheral facial palsy, relapsing orofacial oedema, and a fissured tongue; the most recent review published on the topic has reported 30 published patients. The aetiology of this disease is sti...
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Melkersson-Rosenthal syndrome (MRS) was first described and named after E. Melkersson in 1928 and C. Rosenthal in 1931. MRS is a rare cause of recurrent facial nerve palsy and can manifest as facial paralysis, orofacial edema, and/or tongue fissuring. Presenting with the complete triad, it was scarcely reported in literature. However, the patient r...
Citations
... The onset of the disease is most common in young adults, especially in the second and third decades of life, slightly more common in women than in men [2,3]. The etiology of MRS is not fully understood, but it is postulated to be influenced by various factors, including genetics, infections and allergic factors [4][5][6]. ...
Introduction
Melkersson Rosenthal syndrome (MRS) is a disease of multifactorial origin typically presented with a triad of symptoms including peripheral facial nerve paralysis, plicated tongue and orofacial edema. Diagnosing MRS requires the exclusion of other granulomatous diseases and the correlation of clinical with histopathological finding.
Case presentation
We present the case of a 56-year-old female with a four-month history of lower lip and right mandible angle swelling together with a plicated tongue that appeared during COVID-19 infection. The patient was successfully treated with intralesional Triamcinolone Acetonide at a dose of 40 mg.
Conclusion
The presented case is specific by its late onset since the patient experienced their first symptoms in fifties, which differs from the majority of cases where the diagnosis is usually established in young adults. Infectious factors are established as possible etiologic factors of MRS, but few cases are described to be triggered or worsened by COVID-19 infection.
... The diagnosis is typically delayed, with a median period between 4 and 9 years. [2,4,5,6,7] The purpose of this article is enhancing understanding of this rare neurological illness among healthcare professionals, researchers, and those afflicted by the syndrome. It also aims to emphasize obstacles in diagnosis and management, address novel medications and research possibilities, and encourage a comprehensive approach to care for patients with Merkelson Rosenthal Syndrome. ...
... The cause of Merkelson Rosenthal Syndrome (MRS) is unknown. HSV1 infections, hereditary granulomatous disorders, mycobacterial infections (such as tuberculosis and leprosy), chronic infections, Down's syndrome, psoriasis, thyroiditis, multiple sclerosis, keratitis, Wegener's granulomatous, diabetes mellitus, sarcoidosis, ulcerative colitis, and allergic disorders have all been linked to the etiopathogenesis of this rare pediatric disorder [2,5,11,12]. ...
... In some cases, additional investigations may be considered. These may include TB assessments, karyotyping for Down's syndrome, HLA testing for ulcerative colitis and Crohn's disease, C1 inhibitor deficiency testing for hereditary angioneurotic edema, and, if necessary, next-generation sequencing for genetic analysis [1,2,5,14] It is crucial to note that the selection of investigations may differ depending on specific patient features and the judgment of healthcare providers. As a result, talking with a healthcare practitioner or specialist is critical in determining which investigations are most suited for each patient. ...
Melkersson-Rosenthal Syndrome is an uncommon condition characterized by orofacial edema, facial nerve palsy, and tongue furrowing. The etiology of this condition is uncertain. Corticosteroids, such as intralesional triamcinolone acetonide, are the principal therapy choices for orofacial edema, offering only symptomatic relief. In circumstances when triamcinolone acetonide is not accessible, intralesional betamethasone and oral doxycycline can be used. This thorough review article seeks to improve understanding of Melkersson-Rosenthal syndrome among healthcare professionals, researchers, and those afflicted by the illness. It addresses the many management options used in the treatment of this unusual condition, focusing on the difficulties in diagnosis and management.
... Unfortunately, MRS can show as a mono-or oligosymptomatic disorder, in fact, in most cases, the MRS syndrome often does not present the classic clinical triad, which is why it is difficult to diagnose. [7][8][9] In the literature, several cases of manifestation of MRS with gingival onset are reported, in the absence of local and systemic signs and symptoms. 10,11 In fact, the oral cavity can often be a sign of pathologies. ...
... The site it hits the most is the upper lip, then it can affect the lower lip, the cheek, the buccal mucosa, the eyelid and can even have a wider involvement of the whole face of the patient. 6,9 In the event of only interest of the lips, it can be referred as cheilitis granulomatosa (CG). It is well supported from literature that the edema characterizing OFG and CG is typically F I G U R E 1 Scrotal or fissured tongue or lingua "plicata": considered as a developmental malformation, it can be idiopathic or, in presence of the other two symptoms, a characteristic of MRS. ...
... According to data found in literature, facial nerve paralysis occurs in 30-80% of patients. 6,9 Clinically, it can be confused with Bell's palsy, and this takes to a misdiagnosis of MRS. Facial nerve palsy presents more often as a unilateral and relapsing, partial or complete paralysis of the seventh nerve. ...
Key Clinical Message
Melkersson–Rosenthal syndrome (MRS) remains an enigmatic pathology due to an unknown etiology. Our report, of a 69‐year‐old man with MRS misdiagnosed for about 40 years, underlines the diagnostic difficulty of this condition. A holistic view of the patient, with a correct medical history investigation, are often decisive in the diagnosis of MRS.
Abstract
Melkersson–Rosenthal syndrome (MRS) is a rare disorder with a still unknown etiology. It is defined by three main symptoms, which are orofacial granulomatosis (OFG), facial palsy, and fissured tongue. It generally presents in young people, during the second or third decade, and its incidence in the entire population is about 1%. We focus our attention on a 69‐year‐old man who came to us with an important swelling of the upper lip. His anamnesis revealed that he suffered from a facial palsy four times in his life and at the physical examination we attested the presence of scrotal tongue. We suspected a misdiagnosed MRS and we searched the web in order to give him a diagnosis and a therapy. We found that OFG is the most common symptom of MRS and that it can show as a non complete form, where the three main symptoms cannot occur simultaneously. We also prescribed a therapy based on the use of topic steroids and antiviral, according to literature. After the positive response to the therapy and according to data found in the most recent literature, we can assume that our patient suffers from a misdiagnosed MRS for about 40 years.
... Melkersson-Rosenthal syndrome (MRS) is an unusual manifestation of head and neck defined as a neuromucocutaneous disorder of unknown etiology [1,2], characterized by a symptom triad defined by the presence of recurrent orofacial edema, fissured tongue, and recurrent facial paralysis [1,[3][4][5]. The onset of symptoms is most commonly between 25 and 40 years (range 1-69 years), it is rare in children, and only a few cases have been described in the literature [6,7], the incidence around 0.08% in the general population, and is found more often in females (sex ratio of 2:1) [5][6][7][8][9][10][11]. ...
... Melkersson-Rosenthal syndrome (MRS) is an unusual manifestation of head and neck defined as a neuromucocutaneous disorder of unknown etiology [1,2], characterized by a symptom triad defined by the presence of recurrent orofacial edema, fissured tongue, and recurrent facial paralysis [1,[3][4][5]. The onset of symptoms is most commonly between 25 and 40 years (range 1-69 years), it is rare in children, and only a few cases have been described in the literature [6,7], the incidence around 0.08% in the general population, and is found more often in females (sex ratio of 2:1) [5][6][7][8][9][10][11]. ...
... Therapy of MRS remains difficult and systemic steroids are the most commonly administered [7,14] followed by surgery [10,14] and antibiotics. A great number of published reports didn't mention a therapy given in cases of MRS, therefore no treatment was administered in nearly 40 % of cases. ...
The Melkersson-Rosenthal syndrome constitutes a rare manifestation characterized by a triad of signs and symptoms: recurrent orofacial edema, fissured tongue, and recurrent facial paralysis. The difficulty in diagnosing Melkersson-Rosenthal syndrome is that orofacial edema is common to various diseases besides the lack of awareness of the syndrome by health professionals and the frequent metachronous manifestation of its symptomatology. The aim of this report is to present a classical case of Melkersson-Rosenthal syndrome and its clinical and therapeutic approach. A patient who sought for assistance at the Stomatology Clinic presented a synchronous manifestation of the triad: a left lip and cheek nonpitting edema accompanied by facial paralysis on the same side and fissured tongue. Melkersson-Rosenthal syndrome was diagnosed due to the presence of the triad of signs and symptoms after initially ruling out Crohn’s disease, Sarcoidosis, and tuberculosis due to a lack of intestinal or respiratory complaints and absence of other clinical evidence. The treatment administered was steroids, the most common treatment with a satisfied prognosis we found in the literature for Melkersson-Rosenthal syndrome patients. We recommend its implementation intralesional injections of betamethasone dipropionate as after four infiltrations the edema subsided by 80% with no further relapses within one-year follow-up.
Indexing terms
Facial paralysis; Fissured tongue; Melkersson-Rosenthal syndrome
... MRS is an extremely rare disease and there is little evidence of racial distribution [8]. However, it does appear to affect females three times as often as males [9]. ...
... Several stressors have been identified for certain conditions, such as psoriasis in Crohn's disease [18]. Although the underlying mechanisms that explain the association between psychological stressors and MRS are unknown, they are likely mediated by psychological, neurological, and immunological processes [8]. ...
Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous condition that presents with orofacial swelling, facial paralysis, and a fissured tongue. These classic triad of symptoms, however, very rarely present simultaneously. The symptoms are often seen alone or in pairs and appear at any stage in life. Although the etiology of this condition is unknown, various contributing factors have been suggested including infections, immune deficiencies, stress, and genetic predispositions. We present a case of a 23-year-old female patient who has a longstanding history of MRS, anxiety, and depression, and who attempted to overdose on prescription medications due to suicidal ideations.
... Лечение СМР в основном заключается в назначении системных кортикостероидов и приводит к улучшению состояния у 50-80% пациентов и к снижению частоты рецидивов на 60-75% [9]. Однако рандомизированых исследований, позволяющих определить продолжительность применения кортикостероидов, не проводилось [4]. ...
Melkersson – Rosenthal syndrome (SMR) is a disease manifested by a triad of symptoms: swelling of the lips and/or face, paralysis of the facial nerve, scrotal tongue. In clinical practice, the monosymptomatic variant of SMR is more common. The disease can develop at any age, but most cases occur at 25–40 years with a 2-fold predominance of women among patients. In children, SMR is more often observed at the age of 7 to 12 years, and the smallest child is described at the age of 22 months. The causes of the Melkersson – Rosenthal syndrome have not been definitively clarified. There are many hypotheses about the pathogenesis of SMR, the main of which are allergic, angioedema, infectious and genetic, immunological dysfunction. There are various historically established terms: Mischer’s granulomatous cheilitis, orofacial granulomatosis, SMR, which are regularly used as synonyms in the available literature. To date, SMR remains a diagnosis of exclusion, which is accompanied by the establishment of controversial diagnoses in practical healthcare. In most cases, the treatment of SMR is ineffective. Mainly systemic glucocorticosteroids, antibiotics, antihistamines, immunosuppressive drugs are used, upon discontinuation of which the disease often recurs. In this publication, we present two clinical cases of SMR. The interest of the first case lies in the presence of the entire group of clinical symptoms and the achievement of a positive treatment result. The second case is characterized by the achievement of long-term remission as a result of combination therapy with betamethasone, azithromycin and ketotifen. An overview of successful treatment options is also presented.
... Melkersson Rosenthal syndrome (MRS) is a rare disease present with skin, mucocutaneous and neurological involvement, mostly in young females. [1][2][3] In total, 8-25% of the cases show the complete classical triad, while the majority present as oligo or monosymptomatic forms. [4] While the aetiology of the disease is unknown, certain genetic factors, alterations in immune functions, infections, and allergic reactions have been postulated. ...
... There is a genetic linkage of the syndrome complex. [1,2] Xu et al. [8] identified mutations of FATP1, a fatty acid transport protein, as a causal gene for MRS in a Chinese family. Infectious agents like Mycobacterium tuberculosis, Candida albicans, as well as allergies to food, have been suggested as aetiological factors. ...
... It has a clinical triad of recurring facial paralysis, swelling of face, lips, and presence of furrowed tongue. [1,2] The clinical presentation may be varied, and the patient can present with mono or oligo symptoms or with associated ocular features. The most common mono symptomatic form of MRS is painless recurrent orofacial oedema, called Miescher MRS or cheilitis granulomatosa of Miescher. ...
Melkersson Rosenthal syndrome (MRS) is a very rare neuro-mucocutaneous disease, the aetiology of which is not well defined. It is classically characterised by recurring facial paralysis, swelling of face, lips and presence of a furrowed tongue. The presentation is usually oligosymtomatic, with only about one-fourth of cases showing the classical triad. Here, we present a 32-year-old female with MRS, showing the complete clinical triad of symptoms. We report for the first time, an association of MRS with bilateral optic nerve involvement. A standard criterion for diagnosis and management is lacking. It is important to increase awareness of such a rare syndrome with various associated manifestations, so that it can be accurately identified and treated.
... Unfortunately, MRS can show as a monoor oligosymptomatic disorder. According to Mansour et al. (13) and Ozgursoy et al. (12), only in 8 -18 percentage of cases the complete triad of symptoms occurs, percentage that increases up to 25 according to other authors (8,9). The most frequent symptom is orofacial granulomatosis (OFG), which is reported in 80 -100% of MRS diagnosis (6) . ...
... OFG is defined as an uncommon and chronic inflammatory condition of unknown etiology (7) that affects intra and extraoral mucosal tissues of the head and neck. The site it hits the most is the upper lip, then it can affect the lower lip, the cheek, the buccal mucosa, the eyelid and can even have a wider involvement of the whole face of the patient (6,9). In the event of only interest of the lips, it can be referred as Cheilitis Granulomatosa (CG). ...
... MRS can show as a oligosymptomatic form too, and in this case, facial palsy is the second more common symptom. According to data found in literature, facial nerve paralysis occurs in 30 -80 % of patients (6,9,12). Clinically, it can be confused with Bell's palsy, and this takes to a misdiagnosis of MRS. ...
Melkersson–Rosenthal Syndrome(MRS) is defined by three symptoms: orofacial granulomatosis(OFG), facial palsy and fissured tongue.We focus our attention on a patient who came to us with a swelling of the lip.We suspected a MRS. After the positive response to the therapy, we can assume that he suffers from a MRS.
... Congenital anomalies, such as ankyloglossia, may present a heavy challenge for the parents depending on its level since it can impede normal feeding from an early age or later on when it poses a great difficulty for the child due to the restrictions in normal speech [1,4]. Developmental anomalies should be detected as early as possible since, although asymptomatic, they may predispose the child to develop different complications such as infections or indicate the presence of certain syndromes such as Down syndrome, which is often coupled with a fissurated tongue [2,5]. Many hereditary diseases show first symptoms in the oral cavity. ...
... Developmental anomalies are relatively common in children [1,11]. Although their cause is unknown, they can be caused by hereditary factors or occur as a symptom in various syndromes [2,5,10]. ...
... It is not uncommon to find smaller and shallower furrows originating from the main fissure and spreading radially [2,10,11,35]. Although it represents a more frequent finding in adults, fissured tongue may appear in children in the form of an isolated developmental anomaly or coupled with other disorders such as Down syndrome and Melkersson-Rosenthal syndrome [2,5,10,11,35]. Melkersson-Rosenthal syndrome, although extremely rare in everyday clinical settings, is characterized by a triad of symptoms-orofacial edema, hemifacial paralysis, and fissured tongue [5,39]. Fissured tongue is often related to complications in the form of inflammation and secondary fungal infections as a consequence of food debris retention in deeper grooves [2,10,11,35,39]. ...
Childhood diseases are a continuous source of interest in all areas of general and dental medicine. Congenital, developmental, and hereditary diseases may either be present upon birth or appear in early childhood. Developmental anomalies, although often asymptomatic, may become grounds for different infections. Furthermore, they can indicate certain systemic disorders. Childhood age frequently brings about benign tumors and different types of traumatic lesions to the oral mucosa. Traumatic lesions can be caused by chemical, mechanical, or thermal injury. Mucocele and ranula are, by definition, traumatic injuries of the salivary glands or their ducts. Recurrent aphthous lesions are the most common type of ulcerations in childhood, and their etiology is considered multifactorial. Oral mucosal lesions in children require different treatment approaches depending on etiological factors and clinical presentation. Clinicians should have adequate knowledge of oral anatomy in order to diagnose and treat pathological conditions.
... On the other hand, MRS swelling, which is the most common and major feature of MRS, may be the only symptom occurring. It is painless, non-pitting and may be seen in various facial regions such as the lips, one or both cheeks, eyelids, or rarely, one side of the scalp [7][8][9]. In the first occurrence, swelling may resolve in a few hours or days, but in subsequent episodes, it may last longer and in some cases be able to become permanent. ...
... MRS etiology is not clear. Its etiopathogenesis has been suggested to be associated with many other conditions such as viral infections (herpes simplex virus-HSV1), mycobacterial infections (tuberculosis, leprosy), hereditary granulomatous disorders, Down's syndrome, psoriasis, thyroiditis, multiple sclerosis, keratitis, diabetes mellitus, sarcoidosis, Crohn's disease, ulcerative colitis, and allergic disorders [8,9,11]. However, no strong points exist to explain the etiopathogenesis of MRS. ...
... To prevent these complications, corticosteroids were traditionally prescribed. There is evidence to believe that corticosteroids lead to 50-80% of cases improvement and reduce relapse occurrence by 60-75% [9]. Brief courses of immunosuppressants, non-steroidal anti-inflammatory drugs, antihistamines, and antibiotics were also prescribed depending on the case [3]. ...
Melkersson-Rosenthal syndrome (MRS) is an uncommon neuro-mucocutaneous disease, clinically characterized by a triad of recurrent facial palsy, orofacial swelling, and fissured tongue. This report presents the case of a 38-year-old female diagnosed with MRS based on its three clinical features. A corticosteroid (1 mg/kg/day of oral prednisolone) was prescribed for a week, and then tapered off over two weeks by gradually lowering the dose. Regular annual long-term follow-ups were requested to monitor the disease activity.
