Melkersson-Rosenthal Syndrome Research Articles

Granulomatous cheilitis: a rare case report

Granulomatous cheilitis is a rare granulomatous disorder characterized by a recurrent firm swelling of one or both lips. This is called the cheilitis granulomatosa of Miescher (CGM) when it occurs in isolation. It is called Melkersson-Rosenthal syndrome (MRS) if the classical triad of recurrent or persistent orofacial edema, plicated or fissured tongue (lingua plicata), and relapsing peripheral facial nerve paralysis is present. A 40-year-old female patient came to the Dermatology Department with persistent painless swelling of both lips and perioral area for the last one year. On histopathological examination there was a nodular tuberculoid granulomatous inflammation in patchy pattern present throughout the submucosa. The granuloma consists of lymphocytes, histiocytes and occasional plasma cells. AFB stain and PAS stain were negative. Diagnosis of granulomatous cheilitis was made and patient was put on tab clofazimine and intralesional corticosteroids. The case is being reported due to its rarity and the role of dermatopathologist to make a diagnosis.

Combined wedge resection for lower lip debulking in Melkersson–Rosenthal syndrome

Combined wedge resection for lower lip debulking in Melkersson–Rosenthal syndrome

From Headache to Melkersson-Rosenthal Syndrome: A Case Report

Melkersson-Rosenthal syndrome is described as a syndrome which associates three cardinal signs: peripheral facial paralysis, facial oedema, and fissured tongue. These signs are rarely all present. A range of other symptoms are associated with its, making the diagnosis difficult. We report a case of a twenty-five-year-old women presented recurrent facial paresis and clinical dry syndrome since several years. The facial paresis was secondarily accompanied by headache. After a complete dermatological examination, a Melkersson-Rosenthal syndrome has been suspected. Several treatments have been tried, reducing the symptoms, without making them disappear. The Melkersson-Rosenthal syndrome is well known, but the characteristic triad is rarely present in its entirety. This syndrome is accompanied by a multitude of other symptoms of neurovegetative origin for the most part, and sometimes with autoimmune disorders, which can induce diagnostic delay and/or misdiagnosis.

Miescher’s cheilitis granulomatosa: a case report

Miescher’s cheilitis granulomatosa (MCG) is a rare idiopathic chronic persistent painless lip swelling with granulomatous infiltrate. It is often associated with orofacial granulomatosis and Melkersson—Rosenthal syndrome. Sometimes cheilitis granulomatosa is attributed to sarcoidosis (Besnier—Boeck—Schaumann disease) without systemic lesions. Although there is a possibility that it is a separate disease. Secondary causes include a reaction in response to infection with Mycobacterium tuberculosis, as well as to the introduction of tissue fillers.The publication presents a clinical case of Miescher’s cheilitis, the probable cause of which were the following infectious agents: long­term untreated caries of many teeth and COVID­19.Patient K., 46 years old, complained of swelling and enlargement of the lower lip, a feeling of numbness and discomfort. She fell ill a week ago. Swelling formed gradually over 6—7 days without disturbing the general condition; the body temperature was within the normal range. The lips were swollen, increased in size, slightly hyperemic, the lower one was turned outwards. The lips had a soft­elastic consistency and were not painful to palpate. Pain, tactile and temperature sensitivity were preserved. Dermoscopy was performed. White­grey, structureless areas forming a reticular pattern were observed on the entire surface of the examined area. The vessels were not clear, full­blooded, visualised in the surrounding skin at a considerable distance from the lip edge. The patient was prescribed systemic and topical corticosteroids, hydroxychloroquine, and hyaluronidase. The oral cavity was sanitised. During the month of treatment, the swelling of the lips gradually decreased. At the control examination in two months, the patient’s lip size decreased by 2—2.5 times. There was no relapse of the disease.Conclusions. In our opinion, Miescher’s cheilitis granulomatosa is a separate type of cheilitis, probably of autoimmune nature, in the implementation of which bacterial and viral infectious agents play an important role (focal infection, caries, maxillary sinusitis, frontitis, etc.). The recovery of our patient after the sanitation of the infection foci and complex anti­inflammatory therapy is a convincing proof.

Granulomatous cheilitis and lingua plicata – An incomplete triad of Melkersson–Rosenthal syndrome

Granulomatous cheilitis is a rare granulomatous inflammatory disorder characterized by chronic swelling of the lips. It is an oligosymptomatic form or an incomplete form of Melkersson–Rosenthal syndrome, which comprises recurrent orofacial edema, facial nerve palsy, and fissured tongue. Here, we present a case of a 45-year-old female with persistent upper lip swelling for three months.

Неврологическая патология и симптоматика при некоторых кожных заболеваниях

This article examines two conditions in which neurological symptoms prominently manifest alongside dermatological manifestations. The first condition, Rosolimo–Melkersson–Rosenthal syndrome, encompasses three cardinal features: macrocheilia, fissured tongue, and facial nerve palsy, with macrocheilia often presenting as the monosymptom. When the clinical presentation is limited to macrocheilia, dermatologists typically manage the treatment, while cases with evident facial nerve palsy are referred to neurologists. The second condition is herpes zoster, the clinical manifestations of which unequivocally necessitate dermatological intervention. Apart from cutaneous eruptions, the primary focus shifts to neurological involvement, presenting as neuropathic disorders (agonizing pain syndromes concurrent with eruptions) and post-herpetic neuralgia, which can endure for an extended period. Consequently, the management of such patients involves collaboration between dermatologists and neurologists.

Melkersson-rosenthal sendromu

The Melkersson-Rosenthal syndrome (MRS) is a rare neuro mucocutaneous granulomatous disease presenting with recurrent peripheral facial paralysis, orofacial oedema, and fissured tongue. The coexistence of the classic clinical triad is rare. Clinical findings and examination make the diagnosis. It should be considered in the differential diagnosis of recurrent facial paralysis. In this case report, a 42-year-old female patient with the classical triad of MRS is presented.

Melkersson-Rosenthal Syndrome and Migraine: A New Phenotype Associated with SCN1A Variants?

Peripheral facial palsy rarely occurs as part of Melkersson-Rosenthal syndrome (MRS), which is characterized by the classical triad of tongue cheilitis, recurrent episodes of orofacial swelling, and palsy. MRS is a disorder with variable expressivity and clinical as well as genetic heterogeneity; however, the causative gene remains to be identified. Migraine is a common neurological disorder, presenting with or without aura, which may be associated with neurological symptoms. The classical example of monogenic migraine is familial hemiplegic migraine (FHM), which has phenotypic variability in carriers of variants in the same gene or even carriers of the same variant. We present a family in which two sisters displayed recurrent migraines, one of which presented recurrent facial palsy and had clinical diagnosis of MRS. We performed WES and Sanger sequencing for segregation analysis in the available family members. We identified a c.3521C>G missense heterozygous variant in SCN1A carried only by the affected sister. Variants in the SCN1A gene can cause a spectrum of early-onset epileptic encephalopathies, in addition to FHM; therefore, our finding reasonably explains the proband phenotype, in which the main symptom was recurrent facial palsy. This report also adds knowledge to the clinical spectrum of SCN1A alterations and suggests a potential overlap between MRS and FHM.

Melkersson-Rosenthal Syndrome: A Case Report

Melkersson-Rosenthal syndrome (MRS) is defined by the triad of labial edema, peripheral facial paralysis, and plicated tongue. It is a complex entity with clinical polymorphism and unknown etiopathogenesis. A better understanding of the pathophysiological mechanisms would improve the management of these patients, who often present with significant aesthetic damage. We report a rare case of this syndrome with a plicated tongue and sequellar lagophthalmos.

Melkersson-Rosenthal syndrome - a diagnostic-therapeutic challenge in oral medicine

Melkersson-Rosenthal syndrome (MRS) is a rare disease and is characterised by the classic triad of cheilitis granulomatosa, lingua plicata and peripheral facia- lis paresis. These symptoms rarely manifest themselves all at the same time, often only one of these clinical signs appears. In the literature, the umbrella term orofacial granulomatosis (OFG) is also used, which covers the occurrence of granulomas in the maxillofacial region without associated systemic diseases.

Granulomatous cheilitis of Miescher: a rare entity

BackgroundGranulomatous cheilitis (GC) is a rare entity of unknown etiology. It is a chronic inflammatory disorder with a predilection for young females. It is characterized by asymptomatic unrelenting swelling of lips. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome (a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissuring of the tongue).Case presentationWe herewith report a case of a 27-year-old female, presenting with persistent upper lip swelling, for 3 months. Biopsy from the lesion revealed features of granulomatous cheilitis.ConclusionGC should be considered in the differential diagnosis of unrelenting swelling in the lip. Spontaneous remission is rare, and recurrences are common. Corticosteroids used for treatment provide temporary improvement.

OROFACIAL GRANULOMATOSIS – A REVIEW

Orofacial Granulomatosis (OFG) is a rare clinicopathological entity characterized clinically by the presence of continuous enlargement of the soft tissues of the oral and maxillofacial region and histologically by non-caseating and non-necrotizing granulomatous inammation. The term 'orofacial granulomatosis' has been introduced to indicate the group of various disorders, involving Melkersson-Rosenthal syndrome and granulomatous cheilitis and has been noted to be associated with Sarcoidosis, Crohn's disease and infectious diseases such as Tuberculosis. But, various etiological agents such as food additives, dental materials and microbial agents have been recommended in the disease process. Treatment of orofacial granulomatosis is by corticosteroids but it's not so efcient. It is more important to nd the pathogen rst to specify the appropriate treatment line.

The Importance of the Dental Factor in the Pathogenesis of Melkersson-Rosenthal Syndrome

The Importance of the Dental Factor in the Pathogenesis of Melkersson-Rosenthal Syndrome

Photogallery of diseases of the oral mucosa. Part I

Nowadays, more than 300 diseases of the oral mucosa have been established in the scientific literature. Since pemphigus vulgaris and lichen planus can mimic many other skin conditions it could be difficult to make the diagnosis according only to clinical features. Moreover, pemphigus vulgaris is known to be an autoimmune life-threatening disorder which necessarily need to be treated with high doses of systemic glucocorticoids, whereas lichen planus in some cases can be transformed into squamous cell carcinoma.
 The drug associated erythema and the herpes associated erythema multiforme both affect not only the oral mucosa but also the skin, which makes differential diagnosis easier. Nevertheless, in our practice we can encounter only the oral cavity involvement. Previously, StevensJohnson syndrome was considered as severe form of erythema multiforme affecting both the skin and the oral mucosa.
 Additionally, cheilitis can be observed in patients with atopic dermatitis even in the remission stage.
 Furthermore, MelkerssonRosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips, and the development of folds and furrows in the tongue.

Síndrome de Melkersson-Rosenthal: relato de caso clássico

ABSTRACT 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.

Melkersson–Rosenthal Syndrome (MRS): Multiple, Recurring, and Swelling

Melkersson–Rosenthal Syndrome (MRS): Multiple, Recurring, and Swelling

Oral Mucosal Lesions - A Study of 369 Cases.

Diagnosing and treating oral cavity lesions is a challenging task for most of the clinicians due to similar symptoms and clinical appearances. Frequently, histopathology and immunohistochemistry aid in making the diagnosis. The objectives were to describe the clinical features, and histopathological features and systemic association in patients with oral mucosal lesions (OML). A cross-sectional descriptive study was undertaken at a tertiary care centre in patients with OML. A total of 369 cases with OML were included in the study. Males constituted 61.78% of the cases. History of habits such as tobacco, gutka chewing, smoking, and alcohol was given by 32.25%, 29.81%, 26.56%, and 11.38% of cases, respectively. Common symptoms were soreness, burning sensation, oral pain and ulcers. Both oral and cutaneous involvement was seen in 17.89% of cases. Oral lichen planus (oral LP) constituted largest group of patients (21.96%) wherein reticulate type was the most frequent type and buccal mucosa was the commonest site. Oral carcinomas constituted 20.33% of cases followed by infective etiology (11.92%), vesiculobullous group of diseases (10.30%), aphthous stomatitis (8.94%), premalignant lesions (7.05%) such as leukoplakia (3.80%) and submucous fibrosis (2.44%). Histopathology was done in 209 cases. Clinico-histopathological correlation was seen in oral LP (90.27%), oral pemphigus (82.35%), and malignancies (98.66%). Oral LP formed the largest group of cases followed by Oral squamous cell carcinoma (SCC). Several rare conditions, such as Melkersson-Rosenthal syndrome and blue rubber bleb nevus syndrome were also a part of the study. Thorough clinical and histopathological examination in this diverse group of diseases clinches the diagnosis.

Treatment of Orofacial Granulomatosis—9-Month Follow-up: A Case Report

Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher's granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema.

Complete clinical spectrum of melkersson–Rosenthal syndrome with bilateral temporal optic disc pallor – A case report

Complete clinical spectrum of melkersson–Rosenthal syndrome with bilateral temporal optic disc pallor – A case report

Peripheral facial nerve palsy in children: clinical manifestations, treatment and prognosis.

Sudden onset of unilateral weakness of the upper and lower muscles of one side of the face is defined as peripheral facial nerve palsy. Peripheral facial nerve palsy is often idiopathic and sometimes it could be due to infectious, traumatic, neoplastic, and immune causes. This study aimed to report the clinical manifestation, evaluation, and prognosis in children with peripheral facial nerve palsy. 57 children under 18years of age diagnosed with peripheral facial nerve palsy at Çukurova University, Balcalı Hospital, between January 2018 and September 2021, were included in the study. The mean age of the children at the time of diagnosis was 9.6 ± 7, 4years. Thirty-two (56.1%) of the patients were female and 25 (43.9%) were male. A total of 57 patients were diagnosed with peripheral facial nerve palsy and categorized into many groups by etiology: idiopathic Bell's palsy in 27 (47.5%), infectious in 11 (19.2%), traumatic in 6 (10.5%), and others (due to congenital, immune, neoplastic, Melkersson-Rosenthal syndrome, drug toxicity, and iatrogenic causes) in 13 (22.8%). Forty-six of the children achieved full recovery under oral steroids within 1-7months. Four patients with acute leukemia, myelodysplastic syndrome, Mobius syndrome and trauma did not recover and two patients (schwannoma, trauma) showed partial improvement. Five patients could not come to follow-up control. Peripheral facial nerve palsy is a rare condition in children with different causes. It could be idiopathic, congenital, or due to infectious, traumatic, neoplastic, and immune reasons. So, when a child presents with facial palsy, a complete clinical history and a detailed clinical examination are recommended. Giving attention to the red flag is very important. Peripheral facial nerve palsy in children is considered to have a good prognosis.