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Título

MELKERSSON-ROSENTHAL SYNDROME IN A MALE PEDIATRIC PATIENT: A CASE REPORT

Apresentação do caso

Male patient, 11 years old. The patient presents to the neurology service with right peripheral facial palsy, starting 20 days ago. It started with edema in the face, in the right hemiface, which followed in the later days with pain, cephaelia and right facial paralysis. He also reports that this is the third episode of facial paralysis, always peripheral, all on the right and all with the same characteristics. On a physical exam, it was observed right peripheral facial palsy, lagophthalmic right, lack of creases on the right hemiface and Labial rhyme deviation to the left. Reports family history of seizure, paternal part. Receives diagnostic impression of Melkersson Syndrome CID G51.2, by the findings of Peripheral Palsy of repetition since the 07 months of age and Angioedema. It was prescribed Carmellose sodium eye drops and eye patch to sleep for 10 days and a short cycle with corticoid (40mg). He is currently being followed up at the pediatric neurology service.

Discussão

Melkersson-Rosenthal syndrome is a rare neuromucocutaneous syndrome. It is characterized by the triad of recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue. It often has oligosymptomatic or monosymptomatic characteristics. The clinical diagnosis of this syndrome is suggested by the presence of at least two of the three classic features. In this case, the patient presented: Peripheral Palsy and recurrent orofacial edema. The pathogenesis of this condition is uncertain, which limits the success of treatment. So far there is no known satisfactory treatment. Corticosteroids, non-steroidal anti-inflammatory drugs and lymecycline have been used with satisfactory results with clofazimine, thalidomide, methotrexate and infliximab. The use of prednisone 40mg was prescribed for 5 days, to be used during crises, with no indication of other drugs, aiming at reducing orofacial edema. However, due to the non-occurrence of a more recent consultation, the evolution of the case regarding the drug is not known.

Comentários finais

Melkersson-Rosenthal syndrome is a relevant syndrome for clinical diagnosis, although complementary exams should be suggested to offer a differential diagnosis. There is a shortage of satisfactory treatments given the lack of knowledge about the pathogenesis of this condition. Emphasizing the use of corticosteroids, the most important for controlling the symptoms of this syndrome. Therefore, one should be aware of this pathology, especially in patients with orofacial edema.

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Palavras Chave

melkersson-rosenthal syndrome; pediatric; case report

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