OROFACIAL DISFIGUREMENT IN MELKERSSON-ROSENTHAL SYNDROME: DERMATOLOGICAL AND THERAPEUTIC IMPLICATIONS - A LITERATURE REVIEW

Abstract

Melkersson-Rosenthal Syndrome (MRS) is a rare granulomatous inflammatory disorder known for the triad of recurrent orofacial edema, peripheral facial paralysis, and fissured tongue. However, this complete picture is uncommon, with monosymptomatic or oligosymptomatic forms predominating, which often delays diagnosis and favors structural progression. This study is an integrative literature review that sought to analyze the clinical, histopathological, and therapeutic aspects associated with orofacial disfigurement in MRS. The search was conducted in the BVS, PubMed, and Portal Capes databases, including articles published between January 2021 and January 2026, resulting in 13 selected articles. The results indicate that orofacial edema is associated with progressive tissue remodeling, related to the presence of non-caseating granulomas, chronic lymphoplasmacytic infiltrate, and, in advanced stages, fibrosis. Inflammatory recurrence, coupled with the absence of early diagnosis, favors irreversible lip thickening and a reduced therapeutic response. Intralesional corticosteroids are more effective in early stages, while systemic therapies emerge as possibilities in refractory cases. Despite advances, case reports predominate, and methodological heterogeneity limits therapeutic standardization. Therefore, early diagnosis linked to immunomodulatory interventions becomes fundamental to modifying the evolutionary course of the disease, and further studies are needed to strengthen the evidence.