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Abstract Background Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder characterized by recurrent edema, facial palsies, and nerve dysfunctions often associated with the plicata tongue. Although the etiology of MRS is not well understood, there is growing evidence suggesting an autoimmune involvement. Case presentation This paper presents a case report of a 25-year-old male with MRS as the initial symptom, followed by temporomandibular joint osteoarthritis (TMJ-OA). A comprehensive diagnosis and multidisciplinary treatment approach including surgery, local injections, and oral medication were implemented, resulting in a favorable prognosis. Conclusions These findings support the hypothesis that MRS is a systemic granulomatous disease caused by autoimmunity, which may also influence the occurrence and development of TMJ-OA through immune-related mechanisms. This study emphasizes the significance of systemic immune regulation in the treatment of patients with MRS and TMJ-OA comorbid conditions.

Background: Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder characterized by recurrent edema, facial palsies, and nerve dysfunctions often associated with the plicata tongue. Although the etiology of MRS is not well understood, there is growing evidence suggesting an autoimmune involvement. Case presentation: This paper presents a case report of a 25-year-old male with MRS as the initial symptom, followed by temporomandibular joint osteoarthritis (TMJ-OA). A comprehensive diagnosis and multidisciplinary treatment approach including surgery, local injections, and oral medication were implemented, resulting in a favorable prognosis. Conclusions: These findings support the hypothesis that MRS is a systemic granulomatous disease caused by autoimmunity, which may also influence the occurrence and development of TMJ-OA through immune-related mechanisms. This study emphasizes the significance of systemic immune regulation in the treatment of patients with MRS and TMJ-OA comorbid conditions. [ABSTRACT FROM AUTHOR]

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.

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. 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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

Melkersson-Rosenthal syndrome is a rare neuromucocutaneous granulomatous syndrome of unknown etiology characterized by a classical triad of relapsing orofacial edema, recurrent peripheral facial palsy, and lingua plicata. However, the classical triad is seen in only a minority of the cases. The majority of patients present with mono- or oligosymptomatic forms of the disease. Childhood onset of Melkersson-Rosenthal syndrome is extremely rare, and only a few cases have been described in the literature. This paper reports the case of an 8-year-old girl with cheilitis granulomatosa. Two years after the initial presentation, she presented with a unilateral peripheral facial palsy and a diagnosis of Melkersson-Rosenthal syndrome was established. The aim of this paper is to raise awareness among otolaryngologists of this condition in children presenting with orofacial swelling and/or peripheral facial palsy. Since symptoms do not always occur simultaneously, diagnosis often requires repeated follow-ups. Early recognition and a multidisciplinary approach to this condition are crucial to minimize recurrences and prevent orofacial dysfunction and its associated implications for the child's psychosocial well-being. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

Background: Recurrent facial nerve palsy, orofacial edema, and fissured tongue are a triad of manifestations that characterize a rare disorder named Melkersson-Rosenthal syndrome. It is important to consider this syndrome when diagnosing atypical, unilateral, or bilateral facial palsies with characteristics of familial prevalence. There is no established outcome prediction for this disease and the syndrome does not have a specific duration or prospective timeline. Recurrent facial paralysis can require surgery and a multidisciplinary approach with regular follow-up. Case Report: We describe a 38-year-old woman presenting with a third episode of facial paralysis and discuss her pedigree chart and the treatment course chosen. After conservative treatment with oral corticosteroids, antiviral therapy, and motor physical therapy with no significant improvements, the patient underwent facial nerve decompression surgery with outstanding results. Eight months after surgery and intense postoperative physical therapy, the patient improved from grade VI to grade II palsy on the House-Brackmann Scale. The patient's older brother also presented a fissured tongue and had a history of 2 episodes of facial paralysis. The patient's son, mother, and sister also presented tongue fissuring but did not have any other clinical signs of the syndrome. Conclusions: Despite being rare, Melkersson-Rosenthal syndrome is associated with a family inheritance and its diagnosis has prognostic implications. Therefore, it is of the utmost importance to have suspicion of this disorder in order to improve quality of care and target the treatment accordingly. Surgical treatment in these cases seems to be an excellent choice to treat current facial paralysis and prevent further episodes. [ABSTRACT FROM AUTHOR]

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Melkersson‐Rosenthal syndrome (MRS) is a neuromucocutaneous disease of unknown pathogenesis. With this communication, we describe a case of a 26‐year‐old woman with complete MRS in whom Mycolicibacterium fortuitum was detected in the swelling lip biopsy by next‐ generation sequencing. The patient's symptoms were slightly improved after intralesional corticosteroid injection combined with broad‐spectrum antibiotics, while they were significantly improved after further treatment of dental caries and removal of the residual root. This case provides insight into the possible microbial infection pathogenesis of MRS, and M. fortuitum was speculated to be related to granulomatous and neuronal disorders, most probably from odontogenic origin. [ABSTRACT FROM AUTHOR]

Abstract Background Melkersson–Rosenthal syndrome is a rare disease characterized by the triad of recurrent orofacial swelling with facial paralysis and fissured dorsal tongue. Histologically, noncaseating granulomatous inflammation occurs that confirms the diagnosis. Overlaps between granulomatous diseases such as sarcoidosis and Crohn’s disease are described. Systemic corticosteroid therapy is the treatment of choice for acute attacks. Case presentation We here present a case of a 59-year-old White woman suffering from Melkersson–Rosenthal syndrome with a past history of sarcoidosis on therapy with leflunomide in combination with low-dose tacrolimus successfully treated with the anti-leprosy drug clofazimine after failure of systemic steroid therapy. Conclusions We propose clofazimine as an alternative treatment in steroid-refractory cases.

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.

Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by a triad of relapsing or persistent orofacial edema, recurrent lower motor neuron facial nerve palsy and fissured tongue. Acute, painless, non-erythematosus orofacial edema is observed in all patients. We report a case of a 13-year-old girl who presented with a 2-year history of swelling of the upper lip, facial paralysis, and fissured tongue; she was treated successfully with dapsone.

Skin conditions are a common reason for consulting primary health care. The public frequently expects their primary health care providers to be able to recognise and treat common skin conditions with confidence. Primary care providers must be able to identify ‘red flag’ skin conditions that require dermatology referral. However, diagnosis is often delayed due to the non-specific clinical presentation, low incidence, slow evolution and long-term history of such lesions. We report the case of a patient with extramammary Paget’s disease that presented to her primary care provider and subsequently underwent a wide local excision.

Wiesenfeld described orofacial granulomatosis (OFG) as a group of noncaseating granulomatous disorders affecting the different parts of the body including oral and maxillofacial region, which can have variable etiology. The involvement of oral and maxillofacial region can be exclusive or primary before the involvement of other organs. OFG is a multifactorial clinicopathologic disorder, and its clinical manifestation mimics the various systemic conditions making its diagnosis tough for a dentist. Delay in diagnosis and evaluation of OFG may cause indurated, swollen lips that may compromise cosmetic face value, speaking, and eating functions, and other possible systemic granulomatous disorders can be missed. Here, we present a case of OFG with tooth-associated infection without any recognizable systemic cause. This article presents the diagnostic challenges that a dentist may face in diagnosing such cases, and thus treatment planning should be of the multidisciplinary approach. Regular follow-up is very essential for proper care and management.

Introduction: Granulomatous cheilitis (GC) is a rare idiopathic inflammatory disorder characterized by chronic persistent swelling of the lips. Despite a lingering controversy, it remains linked to orofacial granulomatosis and Melkersson–Rosenthal syndrome (MRS). Aim: To systematically review all cases of GC reported hereto from India with the three being reported now, analyzing patients'/disease characteristics, associated diseases, and treatment protocol. Materials and Methods: Using biomedical search engines such as PubMed, Scopus, and Google Scholar, a systemic review of cases/case series reported from India (1994–2018) was undertaken, and data on patients'/disease characteristics, association with other diseases, and treatment extracted discussed and analyzed without subjecting to statistical analysis due to the small population size. Results: The mean age at presentation of these cases (n, 73: Single, 39: Participants of 6 series, 31 plus presently reported, 3) was 29.36 years and at onset was 26.62 years. There was female preponderance of 1.8. Complete triad of MRS was recorded in 8 (10.95%), and oligosymptomatic forms were as follows: GC + facial palsy, 5 (6.85%); GC + fissured tongue, 4 (5.48%); and facial palsy + fissured tongue, 1 (1.37%). Monosymptomatic GC was seen in 55 (75.34%) and upper lip involvement in 29 (39.72%). Conditions associated with GC included Down syndrome, rosacea, granulomatous vulvitis, and pregnancy. None of the cases were associated with Crohn disease and sarcoidosis. Intralesional triamcinolone acetonide (33, 45.21%) was the most common modality of treatment. Conclusion: Cases of GC from India over the past quarter of a century were reported rarely and showed female preponderance. This chronically recurrent entity with variable severity lacks larger studies and randomized controlled trials for optimum treatment.

Melkersson-Rosenthal syndrome (MRS) is a rare condition without any known etiology. It is characterized by the triad facial paralysis, facial swelling, and the development of folds and furrows at the tongue (fissured tongue). This study aims to report a case of a 59-year-old patient complaining about asymmetric eyelid swelling that had started two years before, associated with pain and redness on her right eye, without repercussions on her visual acuity. The patient underwent a skin biopsy of the right eye’s lower eyelid, which was compatible with the monosymptomatic form of MRS. Consequently, five injections of triamcinolone were performed for the period of one year, with gradual and satisfactory improvement. One year after the end of the treatment, the patient returned with recurrence of the swelling, and therapy with triamcinolone associated with oral steroids was started. However, due to the lack of improvement, Tacrolimus ointment 0.03% was initiated. The patient evolved with an important and significant reduction of the eyelid edema, still being followed at the Hospital Federal dos Servidores do Estado do Rio de Janeiro.

Melkersson-Rosenthal Syndrome (also termed “Miescher-Melkersson-Rosenthal Syndrome”) or (MRS), is a rare neurocutaneous disorder characterized by the clinical triad of recurring facial nerve paralysis, swelling of one or both lips and fissural tongue. We report a patient with Melkersson-Rosenthal Syndrome initially referred to Dermatology Department as angioedema. The diagnosis and treatment procedures were presented with special emphasis to the clinical features of this rare condition.

The association between cheilitis granulomatosa and dental infections (dental caries and apical periodontitis) is still not well understood. Herein, we aimed to investigate the association in large hospital cases with cohort controls. Cheilitis granulomatosa cases (n = 181) were retrieved from Peking University Hospital of Stomatology and age‐ and sex‐matched to controls (n = 181). The χ2‐test, Student's t‐test, and Mann–Whitney U‐test were used to compare the differences between groups. The χ2‐test and odds ratio were used to verify if there was an association and risk relationship. The results showed that both dental caries and apical periodontitis were associated with cheilitis granulomatosa (p < 0.001). Individuals with cheilitis granulomatosa had approximately a twofold increased frequency of dental caries than those without cheilitis granulomatosa (104/181, 57.5% vs. 53/181, 29.3%) (p < 0.001). The odds ratio of dental caries occurring in the case group compared to the control group was 3.211. The frequency of apical periodontitis in patients with cheilitis granulomatosa was significantly greater than in those without cheilitis granulomatosa (109/181, 60.2% vs. 28/181, 15.5%) (p < 0.001). The odds ratio was 8.272. Moreover, apical periodontitis was also locationally related to cheilitis granulomatosa (p < 0.001). Collectively, our study showed that the foci of dental infections are associated with cheilitis granulomatosa, suggesting that proper treatment of focal teeth may be important in the management of cheilitis granulomatosa. [ABSTRACT FROM AUTHOR]

Background: Melkersson-Rosenthal syndrome is a rare disease characterized by the triad of recurrent orofacial swelling with facial paralysis and fissured dorsal tongue. Histologically, noncaseating granulomatous inflammation occurs that confirms the diagnosis. Overlaps between granulomatous diseases such as sarcoidosis and Crohn's disease are described. Systemic corticosteroid therapy is the treatment of choice for acute attacks.Case Presentation: We here present a case of a 59-year-old White woman suffering from Melkersson-Rosenthal syndrome with a past history of sarcoidosis on therapy with leflunomide in combination with low-dose tacrolimus successfully treated with the anti-leprosy drug clofazimine after failure of systemic steroid therapy.Conclusions: We propose clofazimine as an alternative treatment in steroid-refractory cases. [ABSTRACT FROM AUTHOR]

Melkersson–Rosenthal syndrome (MRS) is a rare clinical syndrome. The onset of illness usually occurs in the second decade of life. It is characterized by idiopathic facial paralysis and/or fissured tongue with oro-facial swelling, mainly lip edema. The diagnosis is mainly clinical as the biochemical marker is not ascertained. The isolated facial palsy can mimic various other clinical entities including Bell palsy. The treatment guidelines for MRS are not mentioned in the literature, due to the paucity of randomized clinical trials, which could not happen because of its rarity. However, steroids are effective in this condition. This case report highlights the case of MRS with the typical triad of features, which is quite rare.

Miescher’s cheilitis, also known as cheilitis granulomatosa, is an infrequent disease characterized by chronic recurrent swelling of one lip or both lips. It is considered as one of the three main symptoms of the triad of the Melkersson-Rosenthal syndrome, although in many cases it may develop monosymptomatically.The initial management is based on the administration of corticoids, followed in many cases by the use of other systemic treatments. Nevertheless, because recurrence is quite frequent, surgery remains in many cases as the only definitive treatment.In this report we present the case of a Caucasian woman with Miescher’s cheilitis who was successfully surgically managed.

Background: Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10 different Italian centers and summarized the most recent literature data. Methods: A review of patients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG. Results: Thirty‐nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lips, and 28.2% suffered from Melkersson‐Rosenthal syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG. Oral aphthosis and cervical lymphadenopathy were also described. The mean diagnostic delay was 3.4 years. Histological evaluation was performed in 34/39 patients (87.2%); non‐caseating granulomas were found in 73.5% of them. Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%), and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results. Anti‐TNF‐α and anti‐IgE monoclonal antibodies were used in 6 (15.4%) and 1 (2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response. Conclusions: OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial, management is difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establish registry databases and address challenges of long‐term management. [ABSTRACT FROM AUTHOR]

Background: Melkersson–Rosenthal syndrome (MRS) is a rare disease characterized by the triad of granulomatous cheilitis, fissured tongue, and facial paralysis. Publications concerning large series are rare in the literature. Objectives: To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS. Methods: A retrospective records review was performed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis at oral Diseases Clinic of the Department of Dermatology, University of São Paulo, Brazil (2003, 2017). Results: A total of 51 patients were included, mean age at presentation 35.69 years. Four patients were younger than 18 years. The complete triad of was observed in 10 patients. The rare findings of granulomatous blepharitis, gingivitis and palatitis are presented. Comorbidities included Crohn's disease (5 patients), migraine headaches (1 patient) and convulsions (2 patients). Granulomatous inflammatory infiltrate was detected in 31 biopsies. Medical therapies included included oral and intralesional steroids, thalidomide, dapsone, azathioprine, tetracycline, methotrexate, and surgery, with variable responses. Conclusions: Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations. [ABSTRACT FROM AUTHOR]

Melkersson-Rosenthal syndrome (MRS) is a rare neurological condition that includes a triad of symptoms including recurring facial paralysis, orofacial swelling, and fissured tongue. The diagnosis and treatment of this syndrome is difficult since the classic triad is rarely possible to see in its complete form. The etiology of MRS is unknown, but it is thought to be caused by various factors such as infections, genetic predisposition, immune deficiency, food intolerance, and stress. This case report presents a 22- year-old male patient with classical triad of MRS.

Cheilitis granulomatosa (CG) is a rare idiopathic condition with painless lip swelling, characterized by non-necrotizing granulomatous inflammation in the absence of other identifiable causes such as Crohn’s disease, sarcoidosis, foreign body reaction, or infection. CG may precede the presentation of Crohn’s disease after long-term follow-up. Spontaneous remission of CG rarely occurs. To date, given the rarity of CG, there is no gold standard treatment. Recommended treatments are supported by small studies, case reports/series, and expert opinions. Glucocorticoids are the first-line therapy in the acute stages of the disease; however, recurrence commonly occurs. Previously, methotrexate (MTX) showed a beneficial effect on orofacial swelling in a case of CG accompanied by Crohn’s disease. We present a patient with CG without Crohn’s disease. He was treated with oral MTX in combination with intralesional corticosteroid injection on one side of the lip. The injected side showed improvement, while lip swelling on the noninjected area remained unchanged after 3 months of treatment. Therefore, CG is refractory to treatment with MTX from our experience. Further studies regarding the optimum dosage of MTX is needed.

Abstract Background Melkersson–Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. Case presentation A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphospholipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal syndrome, which was confirmed by histologic findings of non caseating granulomas on lip biopsy. Conclusion Melkersson–Rosenthal syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal biopsy to establish a correct diagnosis and prescribe targeted therapy.

The differential diagnosis of angioedema is extensive because several disorders can mi- mic this clinical feature. Here is reported the case of a 12-year-old boy who presented with recurrent self-limited episodes of non-painful, asymmetrical, swollen lips, without urticaria. After about 1 year lips edema became persistent and associated with gingival hypertrophy and bilateral zygomatic edema. After exclusion of the most likely hypothesis, the histologi-cal findings led to the diagnosis of orofacial granulomatosis, which is a very rare disease in adults, and even less frequent in pediatric patients. The Authors emphasize the importance of an adequate differential diagnosis, since the underlying etiology can be a rare disease requiring a specific treatment. [ABSTRACT FROM AUTHOR]

Background and purpose: Melkersson‐Rosenthal syndrome (MRS) is a rare neuro‐mucocutaneous disease. In addition to the traditional clinical triad, there is also a diversity of clinical signs, and it may be related to other systemic diseases. Methods: In the present study, we report a case of MRS with endocrine disorders that exhibits extraordinary therapeutic efficiency by using hydroxychloroquine (HCQ), explore whether there is an internal connection between MRS and endocrine disorders, and discuss the mechanism of the therapeutic efficiency of using HCQ. The hypothesis proposed for the first time is that MRS may essentially be a systemic granulomatous disease. Results: The physical examination revealed orofacial swelling and fissured tongue. The histopathologic examination showed epithelioid granulomas. Combined with the other examination, this case was diagnosed as incomplete MRS. HCQ and local drugs were introduced. The patient achieved clinical recovery and psychological cure by the 18‐week follow‐up, and the 1‐year follow‐up found no reactivation of MRS. Moreover, the levels of cortisol and adrenocorticotropic were within normal ranges. Conclusions: After the drug therapy was targeted at granuloma, not only did all of the symptoms related to MRS disappear, but the endocrine system also returned to normal. It is speculated that the endocrine disorder in this patient may be related to MRS. We further propose the first‐time hypothesis that MRS may essentially be a systemic granulomatous disease. It provides a new medication method with high‐level efficiency. [ABSTRACT FROM AUTHOR]

Keywords: eyelid edema; Melkersson-Rosenthal syndrome; noncaseating granulomas EN eyelid edema Melkersson-Rosenthal syndrome noncaseating granulomas 203 206 4 01/28/21 20210201 NES 210201 INTRODUCTION Melkersson-Rosenthal syndrome (MRS) is a rare, noncaseating granulomatous disorder first described by Ernst Melkersson and Curt Rosenthal in 1928 and 1931.1 It is defined by its classic clinical symptoms of orofacial swelling, facial nerve palsy, and/or furrowed tongue (lingua plicata). Herein, we describe a case of MRS presenting with isolated bilateral eyelid edema. GLO:8E5/01feb21:cup13719-fig-0001.jpg PHOTO (COLOR): 1 Clinical presentation of the patient with bilateral upper eyelid edema gl Microscopic examination of the bilateral upper eyelid skin revealed subepidermal edema with intersecting bands of dense fibrosis (Figure 2). DISCUSSION MRS is a clinical syndrome characterized by recurrent orofacial edema, relapsing facial paralysis and a fissured tongue. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Aim: To report a rare case of cheilitis granulomatosa (CG) with pica disorder presented as chronic persistent lip swelling with an unusual eating disorder., Background: Cheilitis granulomatosa or Miescher's cheilitis is a rare, persistent, or recurring nontender chronic painless swelling of one or both the lips due to granulomatous inflammation, which was first described by Miescher in 1945. It can present as a monosymptomatic form or as a part of other entities of Melkersson-Rosenthal syndrome (MRS)., Case Description: A 14-year-old female patient presented with persistent diffuse swelling of the upper lip for 1 year. She also had an unusual habit of eating clay, mud, and potentially harmful items like flakes of paint for 1.5 years. Clinically, chronic persistent lip swelling involving upper lip and gingival enlargements were present along with systemic manifestations. Patient counseling with combination therapy showed excellent response with no signs of relapse at 6 months of follow-up., Conclusion: Cheilitis granulomatosa is a unique rare inflammatory disorder with many possible contributory factors and exact etiology is poorly understood., Clinical Significance: This case report draws attention to constant exposure to an allergen that could be one of the possible etiological factors to CG secondary to pica eating disorder. Though it is a rare disease, research on the pathogenesis of CG and clinical trials evaluating the efficacy of the various treatments are needed to enhance our understanding of this disease, to improve the quality of life, as patients are very disturbed by the unsightly and distressing nature of macrocheilitis., How to Cite This Article: Dayanarayana U, Shastry SP, Kempegowda RT, et al. Management of Cheilitis Granulomatosa with Pica Disorder: A Rare Case Report. Int J Clin Pediatr Dent 2024;17(4):483-489., Competing Interests: Source of support: Nil Conflict of interest: None Patient consent statement: The author(s) have obtained written informed consent from the patient's parents/legal guardians for publication of the case report details and related images.Conflict of interest: None, (Copyright © 2024; The Author(s).)

Melkersson-Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous syndrome characterized by recurrent facial nerve palsy, swelling of the upper lip, and fissured tongue. Young adults in the second and third decades are more predisposed. We report a case of a 23-years old female Chinese who presented with 5 days history of right facial weakness; her second event in one year (first weakness was on the left). On examination, she had right peripheral facial nerve palsy (House-Brackmann stage IV) and, mild swelling of the upper lip (Image-Arrow A). Her tongue revealed two central furrows. The anterior central furrow (Image-Arrow B) measured about 2cm and the posterior one measured about 1.5cm with lateral grooves and few posterior perpendicular furrows measuring about 1-2 mm in depth. Her complete blood count, blood sugar, urine routine, C-reactive protein, Serum angiotensin-converting enzyme level was all normal. Her brain magnetic resonance imaging revealed no abnormal signals. She was commenced on oral prednisolone 60mg daily for 5 days, acyclovir 800mg 4 times daily for 5 days, Vitamin B capsules and methylcellulose drops to prevent scleritis. She was commenced on physiotherapy with steady clinical improvement. Although fissured tongue is a common clinical condition with a long list of differential diagnosis, the presence of recurrent facia nerve palsy and swollen lip should raise the suspicion of MRS. Other causes of recurrent facial nerve palsy and buccal mucosa involvement like sarcoidosis, system lupus erythematosus, Bechet disease and Crohn disease should be considered.

Miescher’s cheilitis is an inflammatory disorder characterized by chronic lip swelling due to granulomatous inflammation. It is rare disorder first described by Miescher in 1945. It is monosymptomatic form of Melkersson-Rosenthal syndrome. We report a case of Miescher’s cheilitis in a 59-year old lady presented with recurrent swelling and erythema of upper lip and submental area with no features of facial palsy and fissuring of tongue. Laboratory tests including serum complement C3, C4 and C1 esterase inhibitor functional were within normal limits. The biopsy from the upper lip revealed dermal edema, dilated lymphatic channels and multiple granulomas. After exclusion of other causes of orofacial granulomatosis, the diagnosis of Miescher’s cheilitis was made. Patient was treated with oral hydroxychloroquine and topical tacrolimus and mometasone cream with gradual improvement.

Abstract: Orofacial granulomatosis is a nonspecific term that contains a wide variety of granulomatous entities, which share a clinical and histopathological presentation. It manifests as persistent or recurrent orofacial swelling, amongst other findings. Idiopathic orofacial granulomatosis, characterized by an absence of systemic granulomatous disease, is a diagnosis of exclusion. The main differential diagnosis is Crohn's disease. Its pathogenesis is unknown, however, it seems to be immune-mediated. Patch-test sensitivity to multiple allergens is well documented. Currently, therapeutic options consider restrictive diets, topical, intralesional, and systemic agents. First-line therapy is currently a matter of debate. We present a review of the value of diet therapy in this syndrome, along with two illustrative cases.

Background: Melkersson‐Rosenthal syndrome (MRS) is a rare disease characterized by the triad of recurrent orofacial edema, relapsing facial paralysis and plicated tongue. Histopathological features of MRS have not been extensively analyzed. Methods: This study investigated the histopathological aspects of oral lesions from 47 MRS patients. Results: The most common biopsy site was the upper lip, followed by the lower lip, gingiva and palate. The most important findings were ill‐defined and well‐formed granulomas. Lymphoplasmacytic inflammatory infiltrate was seen in early and late stages of MRS. Edema, fibrosis, vasodilatation and congestion were the most common finding in the lamina propria. Gingival and palate exams also demonstrated granulomatous infiltrates. Regarding the evolution time of the disease, we demonstrated that, in initial phases, there is a lymphoplasmacytic inflammatory infiltrates, followed by a granulomatous infiltrate and, subsequently, fibrosis. Conclusion: Histopathological examination of oral lesions is helpful for the diagnosis of MRS; the absence of granulomatous inflammation does not exclude the diagnosis of syndrome. Clinical and histopathological analysis of the rare gingival and palate lesions is important, since all histopathological findings of the disease were detected in these sites. [ABSTRACT FROM AUTHOR]

Wiesenfeld described orofacial granulomatosis (OFG) as a group of noncaseating granulomatous disorders affecting the different parts of the body including oral and maxillofacial region, which can have variable etiology. The involvement of oral and maxillofacial region can be exclusive or primary before the involvement of other organs. OFG is a multifactorial clinicopathologic disorder, and its clinical manifestation mimics the various systemic conditions making its diagnosis tough for a dentist. Delay in diagnosis and evaluation of OFG may cause indurated, swollen lips that may compromise cosmetic face value, speaking, and eating functions, and other possible systemic granulomatous disorders can be missed. Here, we present a case of OFG with tooth-associated infection without any recognizable systemic cause. This article presents the diagnostic challenges that a dentist may face in diagnosing such cases, and thus treatment planning should be of the multidisciplinary approach. Regular follow-up is very essential for proper care and management. [ABSTRACT FROM AUTHOR]

Melkersson-Rosenthal Syndrome (MRS) is a rare disorder consisting of a triad of persistent or recurrent orofacial edema, relapsing facial paralysis and fissured tongue. It is rarely possible to observe all aspects of the classical triad at the same time, since these symptoms may appear in different times of life cycle. The most common symptom is orofacial edema. Although etiology of MRS is unclear, various factors such as infections, genetic predisposition, immune deficiency, food intolerance and stress have been held responsible. MRS is diagnosed based on clinical features. This case report describes a 39 years old male patient with recurrent swelling of the upper lip. Clinical examinations showed classical triad of MRS. The diagnosis and treatment procedures were presented with special emphasis to the clinical features of this rare condition.

Skin conditions are a common reason for consulting primary health care. The public frequently expects their primary health care providers to be able to recognise and treat common skin conditions with confidence. Primary care providers must be able to identify 'red flag' skin conditions that require dermatology referral. However, diagnosis is often delayed due to the non-specific clinical presentation, low incidence, slow evolution and long-term history of such lesions. We report the case of a patient with extramammary Paget's disease that presented to her primary care provider and subsequently underwent a wide local excision. [ABSTRACT FROM AUTHOR]