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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Author(s):
Gavioli, Camila F. B. [1] ; Florezi, Giovanna P. [2] ; Lourenco, V, Silvia ; Nico, Marcello M. S. [1]
Total Authors: 4
Affiliation:
[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
Total Affiliations: 2
Document type: Review article
Source: JOURNAL OF CUTANEOUS MEDICINE AND SURGERY; v. 25, n. 4, p. 390-396, JUL 2021.
Web of Science Citations: 0
Abstract

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)

FAPESP's process: 17/26990-8 - Melkersson-Rosenthal syndrome: epidemiological, histopathological and serological profile and the interface with orofacial granulomatosis diagnosis
Grantee:Marcello Menta Simonsen Nico
Support Opportunities: Regular Research Grants