Queilite granulomatosa associada à síndrome de Melkersson-Rosenthal

Abstract

Mel kersson-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 discus the clinical features of this disease, the treatment, and the importance of corticosteroid therapy in cases of MRS-related facial palsy.

Síndrome de Melkersson-Rosenthal complicada com hemiespasmo facial e hipertensão intracraniana / Melkersson-Rosenthal syndrome complicated with facial hemiaspasm and intracranial hypertension

“…A SMR é caracterizada pela tríade edema orofacial e paralisia facial recorrentes e língua plicada 16 , características presentes no caso. Não encontramos, na literatura, casos associando a SMR com hemiespasmo facial e HIC.…”

“…Salientamos a importância de se insistir na biópsia, no intuito de documentar a síndrome e instituir a terapêutica apropriada, baseada na corticoterapia prolongada. Os casos não responsivos são encaminhados para tratamento cirúrgico de descompressão do nervo facial, bilateralmente 16 . Acreditamos que o ganho ponderal da paciente (IMC de 27,1 a 30,8 após 15 anos de tratamento), consequente ao uso prolongado de corticosteroides, pode ter levado à síndrome de Hipertensão Intracraniana.…”