Diagnosis Of Melkersson-Rosenthal Syndrome Research Articles

Melkersson-Rosenthal Syndrome: A Mysterious Path to Recurrent Facial Palsy

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous syndrome of unknown etiology with chronic and progressive course, and clinical diagnosis is usually by exclusion. Rarely, it presents with the full triad of recurrent or persistent facial swelling, relapsing facial palsy, and a fissured, geographic 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. Although etiology of MRS is unclear, various factors such as infections, genetic predisposition, immune deficiency, food intolerance and stress have been held responsible. 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. The therapeutic options are varied, though most commonly include corticosteroid therapy and nerve decompression surgery

MELKERSSON-ROSENTHAL SYNDROME: A CASE REPORT

A 20-year-old white man was referred to the oral medicine clinic, complaining of an asymptomatic swelling in the upper lip lasting for around 2 years. His medical history included stress-induced gastritis, bronchitis, allergic rhinitis, and episodes of facial paralysis. The extraoral examination showed a large and diffuse swelling affecting the upper lip vermillion and labial mucosa causing facial asymmetry. The lesion was nonpitting, had a rubbery consistency, and presented with a normal temperature. The vermillion border was dried and stretched, with no ulceration. The intraoral examination revealed deep grooves and fissures on the tongue dorsum. A previous biopsy of the lip showed a chronic inflammatory area. Based on the history and clinical features, the diagnosis of Melkersson-Rosenthal syndrome was made. The patient is under treatment with intralesional infiltration of triamcinolone acetonide in the upper lip. Moreover, he was referred for a physician to investigate a possible intestinal involvement. Support: FAPEMIG.

Melkersson Rosenthal Syndrome: a case report and review of the literature

Melkersson Rosenthal Syndrome (MRS) is a rare neurological disorder characterized by swelling of the face, particularly one or both lips (granulomatous cheilitis), facial muscle weakness (palsy) and a fissured tongue. We present a patient with Melkersson Rosenthal Syndrome, highlighting the clinical triad of symptoms and management. A74-year-old Nigerian male presented to the Oral Medicine Clinic of the University of Benin Teaching Hospital for evaluation of right-sided facial numbness, inability to close the right eye, left facial deviation suggestive of a lower motor neuron type facial palsy of 4 days, and painless swelling of the upper and lower lips for seven days. On examination, he was found to have swelling of the upper and lower lips, multiple fissures on the tongue, right facial paresthesia, and an isolated right-sided facial nerve paralysis. He was empirically managed with 50mg prednisone daily for seven days, which was tapered over two weeks and neurobion 1 tablet daily for a month. This resulted in remission of lip swelling, however fissured tongue remained. Two months later, facial deviation had become less apparent. Patient is being followed up in the outpatient clinic. Melkersson Rosenthal Syndrome is a rare disorder with features of facial swelling, facial nerve palsy and fissured tongue. Some affected individuals may have all three of these features and others may have only one or two. The diagnosis of MRS can be made clinically when there is a complete triad of symptoms as reported in our patient.Keywords: Melkersson Rosenthal Syndrome, clinical symptoms, management

Melkersson-Rosenthal Syndrome in Pediatric Age Group

Melkersson-Rosenthal Syndrome (MRS) is a disease characterized by peripheral facial paralysis, facial edema and fissured tongue. Appearance of this triad is rare, generally monosymptomatic or oligosymptomatic involvement is observed and difficulties or delays in diagnosis can occur due to scarcity of the disease. One or two of the findings and presence of cheilitis granulomatosa in biopsy are sufficient for the diagnosis. Melkersson Rosenthal Syndrome should be considered in differential diagnosis of recurrent facial paralysis. Although findings of the syndrome can regress spontaneously or with medical treatment, it can take a progressive course in some patients, requiring surgical treatment (facial nerve decompression). In this article a pediatric case with diagnosis of Melkersson Rosenthal Syndrome is presented.

ALARM BELLS RINGING FOR MELKERSSON-ROSENTHAL SYNDROME?

Melkersson-Rosenthal Syndrome (MRS) is a rare mucocutaneous disorder, characterised by a clinical triad of facial nerve palsy, facial oedema, and tongue furrowing (lingua plicata). We present the case of a...

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.

Obstructive sleep apnea in a patient with the Melkersson–Rosenthal syndrome

Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology. It is characterized by the triad of macrocheilitis, peripheral facial palsy, and lingua plicata. A 48-year-old nonobese man with a diagnosis of MRS and marked macroglossia was evaluated because of clinically suspected obstructive sleep apnea (OSA). Established causes of OSA such as anatomic abnormalities of the upper airways or the facial skeleton were not present in this patient. Furthermore, hypothyroidism and acromegaly were excluded as underlying diseases. Polysomnography revealed moderate-to-severe OSA. As the swelling of the tongue had been unresponsive to immunosuppressive pharmacotherapy and surgery did not seem to be a reasonable therapeutic option, the patient was finally treated by continuous positive airway pressure therapy. To the best of our knowledge, this is the first report of OSA occurring in a patient with MRS.

Ｍｅｌｋｅｒｓｓｏｎ‐Ｒｏｓｅｎｔｈａｌ症候群例

We report a case of Melkersson-Rosenthal syndrome. The case involved an eight-year-old girl. She had experienced left facial paralysis and its spontaneous regression two years before she first came to our clinic with left recurrent facial paralysis and lower lip swelling. Evoked electromyography revealed that the ratio between left and right was 5%. Recovery of facial nerve function was observed after administration of a steroid. Six months later, right facial paralysis occurred, but it disappeared after steroid therapy. Three years after her first visit, right facial paralysis recurred. Because lingua plicata was also exhibited, the diagnosis of Melkersson-Rosenthal syndrome was confirmed. Complete recovery from right facial paralysis was observed after steroid therapy. Although her mother had a history of sarcoidosis, the patient's chest roentgenography, serum angiotensin converting enzyme level, and ophthalmological evaluation for uveitis revealed no signs of sarcoidosis.