Melkersson-Rosenthal syndrome: recurrent facial swelling and food refusal.

This is a case report of a 27-year-old male who presented with recurrent facial swelling and weakness. The clinical evaluation revealed that the patient had Melkersson-Rosenthal syndrome (MRS), a rare neurological disease characterized by the triad of recurrent facial edema, recurrent facial muscle weakness, and a furrowed or fissured tongue. Accurate data regarding the incidence of MRS is challenging to obtain due to the rarity of the disorder, and early recognition and treatment can potentially reduce the probability of permanent disability.

Melkersson-Rosenthal syndrome is a rare disorder characterized by the triad of recurrent or persist orofacial edema, relapsing facial paralysis and fissured tongue. The syndrome does not always manifest in the complete triad and the diagnosis is often difficult. Here, we report a 24-year-old man presented with recurrent orofacial swelling and facial paralysis. The motor nerve conduction test showed reduction of the amplitude of compound muscle action potential of his facial nerve. The magnetic resonance imaging revealed a T1 gadolinium-enhancement along mastoid segment of left facial nerve. The biopsied specimens from the eyelid revealed a non-necrotizing granulomatous lymphohistiocytic infiltration surrounding the lymphatic duct and branches of his facial nerve. Melkersson-Rosenthal syndrome was diagnosed and the orofacial edema and facial paralysis resolved gradually after treatment with corticosteroid and azathioprine.

A 27‐year‐old lady presented with recurrent facial swelling and a scrotal tongue. She later developed a facial nerve palsy completing the diagnosis of Melkerssohn–Rosenthal Syndrome. While under investigation for her facial swelling she was found to have a positive Kveim test which is unusual in this condition.

Juvenile Recurrent Parotitis (JRP) is an inflammatory condition characterized by recurrent parotid gland inflammation episodes of non-suppurative and non-obstructive nature. This is the second most common salivary disorder in childhood. We present a case of a 5-year- old girl with a recurrent facial swelling on the left side. Panoramic x-ray image excluded any dental or osseous involvement, as well as the presence of sialoliths. Ultrasonography showed hyperechoic focal areas with cavitation of the parotid gland corresponding to sialectasis. Sorological tests for infections like HIV, cytomegalovirus, mumps and other chronic diseases were negative. Based on the clinical and laboratorial features, the diagnosis was compatible with JRP. After two months of follow-up, the patient returned without any swelling or symptomatology. Seven months later, the patient returned with a new facial swelling episode, and this time on the right side. Clinicians should be aware of this condition and include in the differential diagnosis of salivary gland diseases of pediatric patients.

There remain few studies describing in detail the early occurrence and long-term progression of clinical manifestations of orofacial granulomatosis (OFG) in a substantial number of patients. The aim of this study was to determine the early and late clinical manifestations of a large case series of patients with OFG. Clinically relevant data of 49 patients with OFG who attended an Oral Medicine unit in the UK were examined retrospectively. The analyzed parameters included occurrence and typology of initial manifestations at onset and with respect to long-term follow-up. Five major patterns of disease onset were observed. Recurrent facial swelling with/without intra-oral manifestations was the single most common presentation at onset followed by intra-oral ulcers, and other intra-oral and neurological manifestations. The majority of patients later developed a spectrum of additional features. OFG results in multiple manifestations at different time points. The disease onset is characterized by manifestations other than facial swelling in about half of affected individuals. However, patients can develop cosmetically unacceptable lip/facial swelling at a later stage. Nearly all affected individuals ultimately develop lip/facial swelling while about half of all patients develop oral ulceration.

Melkersson Rosenthal syndrome (MRS) is a rare disorder of unknown etiology and comprises the triad: orofacial edema, recurrent facial paralysis and lingua plicata. In the current literature confusing heterogeneity exists, mixing together the historically grown terms cheilitis granulomatosa or granulomatous cheilitis, Melkersson Rosenthal syndrome and the umbrella term Orofacial Granulomatosis (OFG). We provide a systematic review comprising all three disease entities of orofacial granulomatosis using the computerized database "Pubmed Medline" entering the keywords "orofacial granulomatosis" (141 references), "Melkersson-Rosenthal syndrome" (207 references), "granulomatous cheilitis" or "cheilitis granulomatosa" (102 references) back to 1956. Full-text journals and case studies were included, and data synthesis was performed individually. Etiology remains unclear for all three disease entities. Etiological relatedness to chronic inflammatory bowel disease is under discussion and effectiveness was found for different treatments, e.g. local triamcinolone injections, antibiotics, surgical interventions, TNF alpha blockers or exclusive enteral nutrition. No randomized controlled trial concerning the therapy of orofacial granulomatosis was found. As a consequence, the therapeutic conclusion is drawn mainly from small case series, thus limiting the evidence of therapeutic interventions. OFG with the sub-entities MRS and cheilitis granulomatosa is an etiological obscure disease process with various possible therapeutic interventions potentially alleviating the disease course but to broaden treatment knowledge further study in randomized controlled trials is needed.

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

Melkersson-Rosenthal syndrome: About a Tunisian family and review of the literature

ImportanceCurrent recommendations envisage early surgical exploration for complete facial nerve paralysis associated with temporal bone fracture and unfavorable electrophysiologic features (response to electroneuronography, <5%). However, the evidence base for such a practice is weak, with the potential for spontaneous improvement being unknown, and the expected results from alternative nonsurgical treatment also undefined.ObjectiveTo document the results of nonsurgical treatment for posttraumatic complete facial paralysis with undisplaced temporal bone fracture and unfavorable electrophysiologic features.Design, Setting, and ParticipantsProspective cohort study recruiting from April 2010 to April 2013 at a tertiary care university hospital. Follow-up continued until 9 months or until complete recovery if earlier. Study group included 28 patients with head injury–associated complete unilateral facial nerve paralysis with unfavorable results of electroneuronography (<5% response) with or without undisplaced temporal bone fracture. Undisplaced temporal bone fractures were documented in 26 patients (24 longitudinal fractures and 2 transverse fractures).InterventionsPatients received prednisolone, 1 mg/kg, for 3 weeks combined with clinical monitoring every 2 weeks and electromyography monitoring every 4 weeks. As per study protocol, surgical exploration was limited to patients demonstrating motor end plate degeneration on results of electromyography, or having no improvement until 18 weeks.Main Outcomes and MeasuresFacial nerve function was evaluated by the House-Brackmann grading system; Forehead, Eye, Mouth, and Associated defect grading system; and the modified Adour system. Observations were completed at 40 weeks.ResultsAmong the 28 patients in the study (3 women and 25 men; mean [SD] age, 32.2 [8.7] years), facial nerve recovery with conservative treatment alone was noted in all patients. No recovery was seen in any patient at the initial 4-week review. The first signs of clinical recovery were noted in 4 patients by 8 weeks, in 27 patients by 12 weeks, and in all patients by 20 weeks. No patient required surgical exploration. At 40 weeks, 27 patients recovered to House-Brackmann grade I/II and 1 patient to grade III. All 24 patients with longitudinal fractures had grade I/II recovery.Conclusions and RelevanceFor undisplaced temporal bone fractures, nonsurgical treatment leads to near-universal recovery to House-Brackmann grade I/II and is superior to reported surgical results. Recovery is delayed and usually first manifests at 8 to 12 weeks after the fracture. In the current era of high-resolution computed tomography, surgical exploration should not be first-line treatment for undisplaced longitudingal temporal bone fractures associated with complete facial nerve paralysis and unfavorable electrophysiologic features.

A triad of facial palsy, orofacial edema, and furrowed tongue constitutes an uncommon condition known as Melkersson-Rosenthal syndrome (MRS). We report on 14 patients with Melkersson-Rosenthal syndrome. Two patients had facial palsy, 12 had orofacial edema, and 1 patient had a furrowed tongue. Nine patients were treated medically. Intralesional steroid therapy had a 75 percent recurrence rate. Systemic steroid therapy resulted in remission in two of three patients. Surgical excision and reconstruction were carried out in five patients with chronic lip or eyelid edema. This provided relief in all cases. The etiology, clinical presentation, and histologic features are discussed. Three illustrative cases are presented. An algorithm is provided that guides the surgeon with regard to both the medical and surgical treatment of the patient with Melkersson-Rosenthal syndrome.

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.

Melkersson-Rosenthal syndrome (MRS) was first described and named after E. Melkersson in 1928 and C. Rosenthal in 1931. MRS is a rare cause of recurrent facial nerve palsy and can manifest as facial paralysis, orofacial edema, and/or tongue fissuring. Presenting with the complete triad, it was scarcely reported in literature. However, the patient reported here had the complete triad. MRS should be considered when facial paralysis is recurrent or when it presents with orofacial edema, and/or tongue fissuring.

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) 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.