Orofacial Edema Research Articles

Melkersson-Rosenthal Syndrome: A Case Report

Melkersson-Rosenthal syndrome (MRS) is an autosomal dominant transmission disease. The gene responsible is localised in “9p11''. MRS is considered as a rare disorder which consists of a triad of recurrent and persistant peripherical facial paralysis, orofacial edema and fissured tongue. MRS is affirmed to be the consequence of herpes simplex infections, genetic predisposition, immune deficiency, monosodium glutamate and stress. However, it's etiology is still considered unknown. By following the development of MRS, we can prevent the patients from developing both of crohn's and sarcoidosis diseases.

Melkersson-rosenthal syndrome associated with 21 trisomy as a differential diagnosis of angioedema

Background The Melkersson-Rosenthal Syndrome (MRS) is a rare disease that often affects young adults and is characterized by the presence of a painless orofacial edema, congenital fissured tongue and recurrent unilateral or bilateral facial palsy. No predilection for sex or race. Although its etiology is unknown it is believed that genetic, vasomotor, infectious and allergic disorders may be associated. The diagnosis can be made by the association of symptoms with lip biopsy, which shows in the initial stage dilated lymphatics with aggregates of lymphocytes, histiocytes and plasma cells, and in the later stages there is granulomatous inflammation, no caseous with Langerhans giant cells. Due to the rarity of MRS, it should always be included in the differential diagnosis: inflammatory pseudotumor, allergic phenomena, Crohn’s disease, hereditary angioedema, sarcoidosis, lymphangioma and hemangioma.

Melkersson Rosenthal Sendromu Tanısı Atlanıyor Mu

Melkersson Rosenthal Syndrome (MRS) is a neuromucocutaneous granulomatous disease characterized by recurrent facial paralysis, orofacial oedema and fissured tongue. The etiopathogenesis is not known yet, but genetic susceptibility, immunocompromised individual, infectious agents, food intolerance, stress are thought to be responsible. Classic triad is seldomly observed. Generally, monosymptomatic or oligosymptomatic findings are seen. The diagnosis of disease is with clinical findings, and histopathology with non-caseous granulomatous cheilitis further supports the diagnosis. We herein report a patient who previously diagnosed and treated as angioedema and cellulitis in different centers, finally diagnosed as MRS with the classic triad; scrotal tongue, recurrent facial paralysis and orofacial edema; and want to call attention to inadvertent physical examination can miss the diagnosis of MRS.

Melkersson-Rosenthal syndrome: a retrospective study of 44 patients

Conclusion: When patients with recurrent facial paralysis are encountered, otolaryngologists should check for fissured tongue, and question those patients about orofacial edema, minor symptoms, and family history. Histologic evidence is not necessary for the diagnosis of Melkersson-Rosenthal syndrome (MRS), while coronary high-resolution CT (HRCT) reconstruction of temporal bone and food allergen detection may be beneficial. Prophylactic decompression of the facial nerve for patients with appropriate electrophysiological indication may prevent further facial palsy attacks. Objectives: The objective of this study was to analyze the clinical features of a group of patients with MRS with major complaints of facial palsy treated at the Department of Otorhinolaryngology, and to comment on MRS from the perspective of otolaryngologists. Methods: A retrospective review of patient database for the last 6 years in the Department of Otorhinolaryngology in Beijing Shijitan Hospital was performed to find patients diagnosed with MRS. Results: A total of 44 MRS patients were included in this study. The mean age at onset was 14.1 years. A total of 13 (29.5%) patients had family history, 17 (38.6%) revealed broadened fallopian canal on coronary HRCT reconstruction of temporal bone, and 20/23 (87.0%) patients showed positive results in food allergen detection. Thirty-one patients accepted subtotal facial nerve decompression and only one patient had facial palsy recurrence on the same side as the operation.

Asymmetric oro-facial angioedema following alteplase for acute ischaemic stroke

We present a patient with asymmetric oro-facial angioedema following thrombolysis for acute ischaemic stroke with serial photographs of this phenomenon. We discuss the mechanism for the development of asymmetric oro-facial oedema following thrombolysis and suggest a management plan.

Melkersson–Rosenthal syndrome: A facial nerve center perspective

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous granulomatous disorder of unknown etiology, characterized by the triad of facial palsy, lingua plicata (fissured tongue), and orofacial edema. Few articles in the literature report series with more than 20 patients or focus on the facial nerve dominant presentation of MRS. We performed a retrospective review of the patients diagnosed with MRS at a university-based Facial Nerve Center. Twenty-one patients were identified from 1971 to 2010. The age of presentation ranged from 22 to 67 years (mean 44.1). Seven (33.3%) were male and 14 (66.7%) were female. All (100%) patients had facial paralysis. Fourteen (66.7%) patients who initially presented with unilateral paralysis subsequently developed metachronous contralateral paralysis (alternating unilateral facial paralysis). One (4.7%) patient had simultaneous bilateral facial paralysis. The number of episodes per patient ranged from 1 to 8 (mean 3.1). Laterality was relatively equal: 35 episodes occurred on the right side and 31 on the left. The patient with most episodes of facial paralysis had four on the left and four on the right (metachronous). This was followed by three patients with six episodes each. The age of first incidence of facial paralysis ranged from 2 to 60 years (mean 34.4, median of 39). The mean interval between episodes was 4.7 years (range 0-30, median 3). Six (28.5%) of the patients reported a family history of MRS. MRS is a rare disease of unknown pathogenesis in which oligosymptomatic forms predominate. Patients with this disease may present to different specialties complaining of different symptoms, and frequently, not all the classic features of the triad will be present. In our series of facial paralysis patients diagnosed with MRS, a higher proportion had the full triad of symptoms than has been previously reported in the literature.

CR0327 Six cases of cheilitis granulomatosa improved after dental treatment

Cheilitis granulomatosa (CG) is characterized by a persistent lip swelling and considered as an incomplete expression of Melkersson-Rosenthal syndrome, a triad of orofacial edema, facial nerve palsy, and fissure on the tongue. Because the etiology of CG is still unclear, the effective treatment has not been defined yet. Although corticosteroids are widely used for the treatment of CG and are thought to be effective, some cases are resistant. Here we report 5 cases of CG, including 3 steroid-resistant cases, successfully improved after treatment of focal dental infection.

Melkersson–Rosenthal syndrome: a review of seven patients

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 difficult to diagnose and treat. A retrospective review of our MRS patients was performed. The medical files, and treatment, radiologic and histopathologic records of these patients were reviewed. The study group consisted of seven male MRS patients. In four patients, the three classical manifestations were present simultaneously. Neuroimaging examinations found facial soft-tissue thickening and swelling in two patients. The indicators of the disease in our patients were as follows: two had family histories, two had elevated levels of protein in the cerebrospinal fluid, one had an increased immunoglobulin G level in the blood and one had reactive arthritis. All patients responded to systemic corticosteroid treatment. We discuss the genetic, infectious and immunologic factors in the etiology of MRS. Neuroimaging examinations were useful in determining the diagnosis and in determining whether the initial treatment of MRS should be with systemic corticosteroids.

Simvastatin: A Risk Factor for Angioedema?

Objective: To report a case of simvastatin-induced angioedema in a patient with near nightly episodes of orofacial angioedema. Case Summary: A 75-year-old African American female presented to the emergency department with recurrent face, lip, and tongue swelling. The patient described frequent episodes of orofacial edema, with 4 emergency department visits over the previous 6 months. Her home medications were reviewed and simvastatin was identified as a possible contributing medication. Simvastatin was discontinued with resolution of the symptoms during hospitalization and a significant reduction in episodes. Discussion: Drug-induced angioedema has been documented with several agents, most commonly angiotensin-converting enzyme inhibitors. The association with different drug classes has led to several postulated pathways for the development of angioedema. Notable mechanisms include mediation by bradykinin, inhibition of arachidonic acid metabolism, and complement activation. Each pathway culminates in increasing vascular permeability causing fluid accumulation in subcutaneous tissues. While statin use has been associated with drug-induced angioedema in postmarketing reports, there are no published cases involving simvastatin. Use of the Naranjo probability scale demonstrated a probable relationship between simvastatin use and the patient's recurrent angioedema. Conclusions: While statin use is not commonly associated with angioedema, clinicians must be aware of this possible adverse reaction. Consideration must also be given to potential drug interactions, increasing the risk of this adverse event.

Melkersson-Rosenthal Syndrome in a Patient with Psoriatic Arthritis Receiving Etanercept

Melkersson-Rosenthal syndrome is a rare granulomatous neuro-mucocutaneous systemic disease that is characterized by relapsing peripheral facial paralysis, orofacial edema and fissured tongue. The disease etiology is still not well known, but it has been hypothesized that a possible role is played by various causal agents such as infectious diseases, genetic causes, allergic conditions, benign lymphogranulomatosis, various associations with other pathological conditions, particularly with immune-mediated diseases and food contact allergies. In this report we describe the case of a woman, 42 years old, with psoriatic arthritis who developed neurological episodes related to MRS after treatment with anti-TNF therapy. This finding further supports the hypothesis that TNF-alpha blockade, and particularly the use of the TNF-alpha receptor, could trigger the development of granulomatous lesions in predisposed patients. The case we report further sustains the importance for the clinician to take into account this potential adverse event in patients receiving anti-TNF-alpha therapies.

Cheilitis granulomatosa: A case report with review of literature

Cheilitis granulomatosa (CG) is a chronic swelling of the lip due to granulomatous inflammation. It is a rare inflammatory disorder first described by Miescher in 1945. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome; a triad of recurrent orofacial edema, recurrent facial nerve palsy and fissuring of the tongue. As the etiology remains unknown, treatment of CG is challenging. We present a case of CG in a 43-year-old female patient secondary to allergy to certain food items. The present case highlights the importance of thorough investigations in the diagnosis of this lesion as the findings mimic many other granulomatous conditions.

The Melkersson–Rosenthal syndrome: a retrospective study of biopsied cases

Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous syndrome marked by the triad of recurrent nonpitting orofacial edema, fissured dorsal tongue (lingua plicata), and lower motoneuron facial paralysis. Large case series including treatment are limited. A retrospective records review was performed for the diagnoses Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis, confirmed by noncaseating granulomas on biopsy, at the Mayo Clinic in Rochester, Minnesota (1979-2009). There were 72 patients [51 women (71 %), mean age at presentation 39 years (range 8-79)] identified with facial edema with noncaseating granulomas on skin biopsy. Lingua plicata occurred in 34 cases (47 %, 95 % confidence interval 35.3-59.3 %). Unilateral or partial facial nerve palsy occurred in 14 cases (19.4, 95 % confidence interval 11.4-30.8 %). Comorbidities among those with facial edema included periodontal disease (n = 10, 14 %), history of allergic disease (n = 10, 14 %), Crohn's Disease (n = 6, 8 %), migraine headaches (n = 5, 7 %), and systemic lupus erythematosus (n = 2, 3 %). There were no patients who had low C1q or C4 levels among those who were tested. Overall, the full triad canonical of Melkersson-Rosenthal syndrome was observed in nine patients (seven female, median age at symptomatic presentation 35 years (range 10-74 years), 13 %, (95 % confidence interval 6.2-22.9 %) with a median time from first symptoms to diagnosis of 4 years (range 1-35). The medication treatments attempted in the nine patients with the full triad of symptoms included non-steroidal anti-inflammatory drugs, oral and intra-lesional steroids, metronidazole, dapsone, acyclovir, methotrexate, and thalidomide with no consistent treatment responses. The Melkersson-Rosenthal syndrome may present over the course of most of the lifespan and may require several years of observation to be diagnosed. Neurologists who observe a combination of facial edema and facial palsy in a patient should consider the diagnosis of MRS and proceed to a diagnostic skin biopsy and a trial of steroid treatment for their patient.

A rare cause of recurrent peripheral facial palsy

Recurrent peripheral facial palsy (PFP) is not uncommon and it is reported in 3 to 15% of idiopathic PFPs. Other etiologies include intracranial compressive tumors, parotid gland tumors or inflammation, recurrent otitis media, head trauma, multiple sclerosis, sarcoidosis, tuberculosis, Lyme disease, HIV, and the rare Melkersson-Rosenthal Syndrome (MRS). The MRS is a non-necrotizing granulomatous disease characterized by PFP, lingua plicata and orofacial edema, even though the classic triad is reported in only from 20 to 30% of the patients1. We describe a [...]

Melkersson‐Rosenthal Syndrome: A Facial Nerve Center Perspective

Summary Objectives Melkersson–Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous granulomatous disorder of unknown etiology, characterized by the triad of facial palsy, lingua plicata (fissured tongue), and orofacial edema. Few articles in the literature report series with more than 20 patients or focus on the facial nerve dominant presentation of MRS. Methods We performed a retrospective review of the patients diagnosed with MRS at a university-based Facial Nerve Center. Results Twenty-one patients were identified from 1971 to 2010. The age of presentation ranged from 22 to 67 years (mean 44.1). Seven (33.3%) were male and 14 (66.7%) were female. All (100%) patients had facial paralysis. Fourteen (66.7%) patients who initially presented with unilateral paralysis subsequently developed metachronous contralateral paralysis (alternating unilateral facial paralysis). One (4.7%) patient had simultaneous bilateral facial paralysis. The number of episodes per patient ranged from 1 to 8 (mean 3.1). Laterality was relatively equal: 35 episodes occurred on the right side and 31 on the left. The patient with most episodes of facial paralysis had four on the left and four on the right (metachronous). This was followed by three patients with six episodes each. The age of first incidence of facial paralysis ranged from 2 to 60 years (mean 34.4, median of 39). The mean interval between episodes was 4.7 years (range 0–30, median 3). Six (28.5%) of the patients reported a family history of MRS. Conclusions MRS is a rare disease of unknown pathogenesis in which oligosymptomatic forms predominate. Patients with this disease may present to different specialties complaining of different symptoms, and frequently, not all the classic features of the triad will be present. In our series of facial paralysis patients diagnosed with MRS, a higher proportion had the full triad of symptoms than has been previously reported in the literature.

Melkersson Rosenthal syndrome: a histopathologic mystery and dermatologic challenge

Melkersson Rosenthal syndrome (MRS) is rare disease of unknown etiology characterized by orofacial edema, facial nerve palsy and fissured tongue. Microscopically, it shows epithelioid non-caseous granulomas; however, edema and perivascular lymphocytic infiltrates have been described. Two different clinical forms of MRS are presented in this report. In the complete form (Case 1), the main histopathologic finding was a non-necrotizing granulomatous inflammation with 56% of the total number of cells composed of B cells (CD 20(+) ) principally located in the granuloma's center and 33% being T cells predominating in the surrounding area, of which 48% were CD 4(+) and 16% were CD 8(+) lymphocytes. In the monosymptomatic form (Case 2), the inflammatory cells were dispersed into the connective tissue without granulomatous formation. B cells were scanty, and 78% of the cells were CD 45(+) T cells, with 46% and 34%, CD 8(+) and CD 4(+) phenotype, respectively. These cases showed different clinical, histopathological and immunohistochemical forms of MRS, suggesting different host immune responses.

Melkersson-Rosenthal Syndrome Presenting as Chronic Eyelid Swelling.

Melkersson-Rosenthal Syndrome (MRS) is a rare disorder characterized by orofacial edema, facial palsy, and fissured tongue. A 64-year-old man presented with a fissured tongue and persistent chronic right upper eyelid edema of 15 years duration. The diagnostic biopsy revealed non-necrotizing granulomatous inflammation adjacent to blood and lymphatic vessels. Characteristic granulomas with focal occlusion of dilated lymphatic channels were present. Special stains for fungi, acid-fast microorganisms and bacteria were negative. Ptosis due to a mechanical effect of the persistent eyelid edema required surgical treatment including debulking and advancement of the levator muscle. At 6 months after surgery the patient showed symmetrical eyelid position without edema. The case demonstrates an uncommon presentation of Melkersson-Rosenthal syndrome with prominent upper eyelid edema and lingua plicata, which was initially misdiagnosed. The clinical findings coupled with the characteristic granulomatous lymphangitis present in the biopsy are crucial for a definite diagnosis.

Melkersson-Rosenthal syndrome and its variants- The Mayo Clinic experience

RATIONALE: Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis are conditions in which patients develop swelling of different parts of the face secondary to non-caseating granulomas. We examined the clinical presentation, therapeutic intervention, and coexisting disorders in those patients. METHODS: The Mayo Clinic database was reviewed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis or orofacial granulomatosis between 1976 and 2009. We reviewed the clinical presentation, laboratory studies and treatment regimens. RESULTS: There were a total of 72 patients who presented with focal facial swelling and had area- specific biopsies that showed non- caseating granulomas consistent with Melkersson-Rosenthal syndrome. There were 51 females and 21 males with a median age of 39 years (range 8 to79 years). The complete triad of Melkersson- Rosenthal Syndrome, which includes orofacial edema, intermittent facial palsy and lingua plicata, was present in seven patients (10%). Orofacial edema was present in all the patients with the lips being the most common area affected (74%). Lingua plicata and facial palsy were present in 47% and 10% of the patients, respectively. Crohn's disease was present in 8% of the patients. None of the patients had sarcoidosis. Two patients had systemic lupus. Only two of the fourteen patients had improvement with antihistamines. About half of those who received oral steroids (n= 25) and about 70% of those who received intralesional steroids (n= 17) had an improvement. Other successful therapies included dapsone, minocycline, or metronidazole. Two patients had cheiloplasty. CONCLUSIONS: In patients with Melkersson-Rosenthal syndrome, oral or intralesional steroids are a management option.

Melkerson Rosenthal Syndrome – A Case Report

Melkersson-Rosenthal Syndrome (MRS) is a rare syndrome consisting of a triad of persistent/ relapsing facial palsy, recurrent orofacial edema and fissured tongue. The syndrome does not manifest in complete triad at all times and diagnosis is often difficult. We present a case of a 40-year-old male with a complete triad of all the manifestation of this syndrome. This case highlights the need to consider MRS as one of the differential diagnosis of recurrent facial palsy and also stressing the importance of a thorough intraoral examination when patients presents with relapsing facial palsy.

Complete Melkersson-Rosenthal syndrome with multiple cranial nerve palsies

Melkersson-Rosenthal syndrome (MRS) is an uncommon, complex neuromucocutaneous disorder characterized by recurrent orofacial oedema, facial palsy and fissured tongue. Complete MRS is uncommon. A case of complete MRS with multiple cranial nerve palsies is reported.

Anesthetic management of patients with Melkersson Rosenthal syndrome

Melkersson Rosenthal Syndrome (MRS) is a rare disorder characterized by relapsing facial paralysis, persistent or recurrent orofacial edema, and lingua plicata. It may cause difficult airway, drug allergy, and angioedema. In our anesthetic management of two patients with MRS, preanesthetic immunological blood examination and skin tests for hypersensitivity to anesthetic drugs were applied. Because the principal goal is to avoid all factors that may stimulate, an allergic reaction, anesthetic drugs known to trigger urticaria were avoided. Body and operating room temperatures, changes of which may trigger allergic reactions, were kept constant during the perioperative period. Emergency precautions were taken for probable angioedema. MRS is a rare syndrome, and if its manifestations are misunderstood as simple facial paralysis, it may be overlooked by anesthesiologists. Anesthesiologists must be careful of several problems in patients with MRS.