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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.

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BACKGROUND: Melkersson-Rosenthal syndrome is a rare disorder that is characterized, in its full form, by recurrent facial nerve palsy, fissured tongue, and orofacial edema. Most cases present as oligosymptomatic or monosymptomatic forms. Its etiology is still unknown and its course is chronic and it may be progressive. CASE REPORT: We present the case of a nine-year-old girl with recurrent episodes of peripheral facial nerve palsy. During the study, lip edema, benign migratory glossitis, and angular cheilitis were observed, which is why a clinical diagnosis of Melkersson-Rosenthal syndrome was made. CONCLUSIONS: This syndrome must be considered in the differential diagnosis with the presence of acute peripheral facial nerve palsy and/or facial edema due to its behavior and progressive evolution.

Antecedentes: El síndrome de Melkersson-Rosenthal es una entidad poco frecuente caracterizada, en su forma completa, por parálisis facial recurrente, lengua fisurada y edema orofacial. La mayoría de los casos se presentan como formas oligosintomáticas y monosintomáticas. Su etiología es aún desconocida y tiene un curso crónico que puede ser progresivo. Caso clínico: Presentamos el caso de una niña de nueve años de edad con episodios recurrentes de parálisis facial periférica. Durante su estudio se observó edema labial, glositis migratoria benigna y queilitis angular, por lo que se formuló el diagnóstico clínico de síndrome de Melkersson-Rosenthal. Conclusión: Debemos considerar este síndrome dentro del diagnóstico diferencial ante la presencia de parálisis facial periférica recurrente o edema facial, debido a su comportamiento y evolución progresiva.

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Melkersson-Rosenthal syndrome (MRS) consists of persistent or recurrent orofacial edema, relapsing facial palsy and fissured tongue. The complete triad of symptoms is uncommon, varying from 8 to 25%. The presentation of only one symptom is more common. The most frequent complaint is facial edema and enlargement of the lips. We describe a case of a 17-year-old Brazilian girl with limited edema of the lower lip and fissured tongue due to MRS. Her complaints had started two years before. She referred previous clinical treatments without success. We proposed intralesional injection of triamcinolone at 20 mg every 15 days associated with oral clofazimine at 50 mg/day for three months. The lip became normal after four triamcinolone injections. Recent studies have considered MRS a granulomatous disease, and possibly the initial presentation of Crohn's disease in orofacial area of some patients. MRS patients, therefore, should be screened and monitored for gastrointestinal symptoms. Corticosteroid treatment seems to be effective in reducing lip enlargement. We discuss the clinical features of this disease, the treatment, and the importance of corticosteroid therapy in cases of MRS-related facial palsy.

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UNLABELLED: The revision of MEDLINE from 1966 to 2003 did not report any association between multiple sclerosis (MS) and Melkersson-Rosenthal syndrome (MRS). This is a case report of a 51-year-old woman, with history of four recurrent Bell's palsies. In 1999 she developed a right facial paralysis due to a supranuclear pyramidal lesion with right monoparesis. The family history showed five relatives with recurrent Bell's paralysis and plicata tongue. PHYSICAL EXAMINATION: right Bell's paralysis, left supranuclear facial paralysis, furrowed tongue, right hemiparesis with pallor of the optic disks. Brain magnetic resonance imaging (MRI) demonstrated the typical lesions of MS and CSF oligoclonal bands. This is the first observation of a patient with hereditary MRS and MS. The link between both diseases is discussed.

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O presente trabalho visa relatar um caso clínico completo de um paciente de 24 anos de idade, feoderma, portador da Síndrome de Melkersson-Rosenthal, atendido no Setor de Cirurgia Buco-maxilo-facial do Hospital Universitário Pedro Ernesto. Atríade clássica desta síndrome, constituída de granulomatose orofacial, paralisia facial (Paralisia de Bell) e língua fissurada, é rara e pode ser observada no presente caso. Este artigo visa também atentar para outras possíveis manifestaçöes orofaciais da síndrome, além de ressaltar os parâmetros para o correto diagnóstico e seus possíveis tratamentos

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Os autores apresentam caso de criança com a Síndrome de Melkersson-Rosenthal caracterizada por paralisia facial periférica, edema recorrente de lábio e língua plicata e fazem revisäo da literatura sobre aspectos fisiopatológicos e de tratamento

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This a case report of a 24 year old black woman who referred to her physician recurrent episodes of inferior lip enlargement for a period of two years. Remission periods were incomplete, unrelated to any apparent cause and plicate tongue. No improvement was seen with medication, including the use of steroids. Complementary exams were negative except the biopsy which reported granulomatous cheilitis. We conclude that the Melkersson Rosenthal Syndrome must be considered when confronted with a patient who suffers from lip enlargement and pleated tongue, with or without neurological alterations as for example, facial paralysis.

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