MELKERSSON-ROSENTHAL SYNDROME: LYMPHOSCINTIGRAPHY-DOCUMENTED IMPAIRMENT AND RESTORATION OF FACIAL LYMPHATIC DRAINAGE IN THE COURSE OF DISEASE

Abstract

Melkersson-Rosenthal syndrome (MRS)is an idiopathic, rare disorder manifested byfacial swelling, congenital plicated tongue andrecurrent peripheral facial nerve palsy. Labialinvolvement alone is referred to as cheilitisgranulomatosa. Differential diagnosis of MRSincludes allergic angioedema, bacterial, viralor filarial infections as well as autoimmunologicalinflammation in the course of systemiclupus erythematosus, dermatomyositis, andothers. We present 4 patients who experiencedperiodically painless edema of the face and/orlips. Lesions were diagnosed as recurrentQuincke’s edema and were treated withantihistamine agents and glucocorticoidswithout improvement.In all four cases of MRS, we were able todocument impaired lymphatic drainage fromthe swollen area using lymphoscintigraphy.We also documented in follow-up lymphoscintigraphya restoration of lymphatic flow inthree of the four patients with MRS and theseresults corresponded with clinical improvement.We have demonstrated that lymphaticpathology plays an important role in pathophysiologyof chronic facial swelling inpatients with Melkersson-Rosenthal syndrome