Clinical Profile of Melkersson-Rosenthal Syndrome/Orofacial Granulomatosis: A Review of 51 Patients.
Melkersson-Rosenthal syndrome
facial paralysis
fissured tongue
geographic tongue
granulomatous cheilitis
orofacial granulomatosis
Journal
Journal of cutaneous medicine and surgery
ISSN: 1615-7109
Titre abrégé: J Cutan Med Surg
Pays: United States
ID NLM: 9614685
Informations de publication
Date de publication:
Historique:
pubmed:
13
2
2021
medline:
15
12
2021
entrez:
12
2
2021
Statut:
ppublish
Résumé
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. To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS. 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). 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. Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations.
Sections du résumé
BACKGROUND
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
OBJECTIVE
To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS.
METHODS
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
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
CONCLUSIONS
Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations.
Identifiants
pubmed: 33573395
doi: 10.1177/1203475421995132
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM