Melkersson-Rosenthal syndrome - delay in the diagnosis of an early-onset oligosymptomatic variant

Fabrícia Carolino; Margarida Fernandes; José Luís Plácido

doi:10.1016/j.pbj.2015.10.001

Melkersson-Rosenthal syndrome - delay in the diagnosis of an early-onset oligosymptomatic variant

Porto Biomed J. 2016 Mar-Apr;1(1):43-45. doi: 10.1016/j.pbj.2015.10.001. Epub 2016 Mar 1.

Authors

Fabrícia Carolino¹, Margarida Fernandes², José Luís Plácido¹

Affiliations

¹ Imunoalergology Department, São João Hospital Center, Porto, Portugal.
² Pathology Department, São João Hospital Center, Porto, Portugal.

Abstract

Melkersson-Rosenthal syndrome is a rare neuro-mucocutaneous disease with a chronic intermittent course, characterized by a classic triad of orofacial swelling, fissured tongue (lingua plicata) and facial paralysis. The authors describe the case of an oligosymptomatic variant (lip and tongue involvement) with childhood onset, whose diagnosis was only established at the age of 19 years. The syndrome's pathophysiology is unclear and the treatment is challenging; corticosteroid therapy is the mainstay of treatment and is associated with clinical and histological improvement.

Keywords: Cheilitis granulomatosa; Lingua plicata; Melkersson-Rosenthal syndrome.