Spontane Zungennekrose bei rheumatoidem Hyperviskositätssyndrom. Fallbericht und Literaturübersicht

Abstract

Because the tongue is an organ known for its excellent blood supply, ischaemic lingual necrosis is extremely rare within clinical everyday life. Acute lingual circulatory disturbances can result from impairment of venous drainage or more often from ischaemic arterial occlusion. Due to permanent function loss of the tongue, apparent lingual necrosis may lead to severe mutilation of the patient. While vasculitis of the lingual arteries in temporal arteritis is said to be the most frequent causation of tongue necrosis, diagnosing the underlying disease of lingual ischaemia may sometimes be challenging for the clinician. We present the first reported case of a spontaneous lingual necrosis in a patient with rheumatoid arthritis, due to polyclonal gammopathy with extensive hyperviscosity syndrome and local vasculitis. Clinical symptoms, diagnosis and therapy of tongue necrosis are presented in a case report. Besides an overview on the disorder of hyperviscosity syndrome, the discussion will illustrate pathogenetic, diagnostic and therapeutic considerations of lingual ischaemia. The international medical literature is reviewed to summarize the causes of tongue necroses that are described up to now. The particular importance of temporal arteritis Horton for the otolaryngologist in general and for the development of lingual necrosis in particular is highlighted. Apart from the presentation of the first reported case of lingual necrosis in rheumatoid hyperviscosity syndrome, the intention of this article is to draw the clinician's attention on the fundamental aspects of lingual ischaemia and of temporal arteritis.