RESUMO
BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of granulomatous cheilitis, fissured tongue, and facial paralysis. Publications concerning large series are rare in the literature. OBJECTIVES: To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS. METHODS: A retrospective records review was performed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis at oral Diseases Clinic of the Department of Dermatology, University of São Paulo, Brazil (2003, 2017). RESULTS: A total of 51 patients were included, mean age at presentation 35.69 years. Four patients were younger than 18 years. The complete triad of was observed in 10 patients. The rare findings of granulomatous blepharitis, gingivitis and palatitis are presented. Comorbidities included Crohn's disease (5 patients), migraine headaches (1 patient) and convulsions (2 patients). Granulomatous inflammatory infiltrate was detected in 31 biopsies. Medical therapies included included oral and intralesional steroids, thalidomide, dapsone, azathioprine, tetracycline, methotrexate, and surgery, with variable responses. CONCLUSIONS: Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations.
Assuntos
Granulomatose Orofacial/diagnóstico , Síndrome de Melkersson-Rosenthal/diagnóstico , Adolescente , Adulto , Brasil , Comorbidade , Feminino , Granulomatose Orofacial/tratamento farmacológico , Humanos , Masculino , Síndrome de Melkersson-Rosenthal/tratamento farmacológico , Estudos RetrospectivosRESUMO
Orofacial granulomatosis is a generic term applied to manifestations of several diseases including sarcoidosis, Crohn's disease, Melkersson-Rosenthal syndrome, cheilitis granulomatosa of Miescher, tuberculosis and foreign-body reactions. What bonds these diseases together is the presence of noncaseating granulomas. A typical clinical manifestation of orofacial granulomatosis is recurrent labial swellings that eventually persist. This article describes 2 cases of OG diagnosed with the aid of immunohistochemical analysis and successfully treated with intralesional steroids.