Melkersson-Rosenthal syndrome treated with sulphasalazine

Abstract

Introduction: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of symptoms labial/facial oedema, fissured tongue and facial palsy. The majority of patients present with an incomplete form, commonly with only labial oedema (Miescher cheilitis). Several treatments have been reported to be effective, but none has proved its efficacy in well-designed controlled trials. OBJECTIVES: As there is frequently evidence of granulomas in lip biopsies and as sulphasalazine is the drug of choice in other granulomatous disorders, such as ulcerative colitis, we proposed to conduct a prospective study of the efficacy of sulphasalazine in MRS. PATIENTS AND METHODS: We included seven MRS patients, five females and two males, with an average age of 30.71 years (SD ± 9.79 years). The average duration of disease was 10 years (SD±8.98 years). Other diseases that can appear as labial oedema were ruled out. We also performed quantitative and qualitative assessment of the enzyme gIucose-6-phosphate-dehydrogenase (G6PD), which was always normal. RESULTS: An improvement was noticed in labial oedema in five patients (71.4%) after an average of 11.4 months of treatment (2-20 months). CONCLUSIONS: Sulphasalazine seems to be an efficient treatment in MRS, although controlled studies are necessary, which also do not exist for the other proposed treatments for this syndrome.

Key Words:

• Melkersson
• Rosenthal syndrome
• Miescher cheilitis
• Sulphasalazine