Articles

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

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Abstract

Melkersson-Rosenthal syndrome (MRS)is an idiopathic, rare disorder manifested by facial swelling, congenital plicated tongue and recurrent peripheral facial nerve palsy. Labial involvement alone is referred to as cheilitis granulomatosa. Differential diagnosis of MRS includes allergic angioedema, bacterial, viralor filarial infections as well as autoimmunological inflammation in the course of systemic lupus erythematosus, dermatomyositis, and others. We present 4 patients who experienced periodically painless edema of the face and/or lips. Lesions were diagnosed as recurrent Quincke's edema and were treated with antihistamine agents and glucocorticoids without improvement.

In all four cases of MRS, we were able to document impaired lymphatic drainage from the swollen area using lymphoscintigraphy. We also documented in follow-up lymphoscintigraphy a restoration of lymphatic flow in three of the four patients with MRS and these results corresponded with clinical improvement. We have demonstrated that lymphatic pathology plays an important role in pathophysiology of chronic facial swelling inpatients with Melkersson-Rosenthal syndrome.

Keywords: Melkersson-Rosenthal syndrome, cheilitis granulomatosis, orofacial granulomatosis, lymphoscintigraphy, facial lymphedema

How to Cite:

Nittner-Marszalska, M., Krasnowska, M., Solarewicz-Madejek, K., Jedrzejuk, D. & Szuba, A., (2010) “MELKERSSON-ROSENTHAL SYNDROME: LYMPHOSCINTIGRAPHY-DOCUMENTED IMPAIRMENT AND RESTORATION OF FACIAL LYMPHATIC DRAINAGE IN THE COURSE OF DISEASE”, Lymphology 43(1), 34-41.