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

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.

Granulomatous Cheilitis: A Case Report

Introduction: Granulomatous cheilitis is a rare condition, with an unknown etiological pathway, resulting in inflammation of the lips. This case report demonstrates the efficacy of intralesional triamcinolone with this persistent disorder.Case report: A 19-year old man presented with orofacial swelling on the right side for approximately 18 months. Previous treatments of minocycline and metronidazole were unsuccessful. The patient has no other symptoms and an unremarkable family history. A regiment of topical anesthetic and intralesional triamcinolone showed significant improvement.Discussion: This rare condition has many possible contributory factors with very few established treatments. Our patient appeared to present either granulomatous cheilitis or monosymptomatic Melkersson-Rosenthal syndrome. Our primary goal was to abate the patient’s symptoms for their comfort, hence the implication of the benzocaine gel.Conclusion: There are various therapeutic methods described, however, we believe intralesional triamcinolone injections with prior application of topical anesthetic to ease discomfort, could be an alternative treatment.

Recurrent granulomatous cheilitis associated with Crohn’s disease successfully treated with ustekinumab: case report and literature review

Granulomatous cheilitis, characterized by persistent inflammation of the lips and a granulomatous histology, is sometimes associated with Crohn’s disease and is a therapeutic challenge. Reported evidence indicates treatment with an anti-TNF agent (mainly infliximab) is the most recommended therapeutic option after failure of conventional treatments. The clinical case reported the effectiveness of ustekinumab, a monoclonal antibody against interleukins 12/23, to induce the remission of severe and recurrent granulomatous cheilitis in a patient with Crohn’s disease.