Clinical Profile of Melkersson-Rosenthal Syndrome/Orofacial Granulomatosis: A Review of 51 Patients

Autor(es)

Gavioli, Camila F. B. ^[1] ; Florezi, Giovanna P. ^[2] ; Lourenco, V, Silvia ; Nico, Marcello M. S. ^[1]

Afiliação do(s) autor(es)

^[1] Med Sch, Dept Dermatol, Ave Dr Eneas de Carvalho Aguiar 255, 3oA, BR-05403000 Sao Paulo, SP - Brazil

^[2] V, Univ Sao Paulo, Dent Sch, Dept Pathol, Sao Paulo - Brazil

• Tipo de documento: Artigo de Revisão
• Fonte: JOURNAL OF CUTANEOUS MEDICINE AND SURGERY; v. 25, n. 4, p. 390-396, JUL 2021.
• Citações Web of Science: 0

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 Sao 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. (AU)

• Processo FAPESP: 17/26990-8 - Síndrome de Melkersson-Rosenthal: Perfil epidemiológico, histopatológico e sorológico e sua interface com o diagnóstico da granulomatose orofacial
• Beneficiário: Marcello Menta Simonsen Nico
• Modalidade de apoio: Auxílio à Pesquisa - Regular