Τρίτη 10 Ιανουαρίου 2017

Amyloidotic pseudo-spectacles

A 60-year-old man presented with progressive exertional dyspnoea and swelling in the bilateral lower limb. His relatives noted discolouration around the eyes over the last 6 months. He had a history of easy bruisability and noticed spontaneous ecchymotic patches for a couple of years with no other significant bleeding manifestations. On physical examination, he was noted to have bilateral periorbital ecchymosis with normal sclera (figure 1), raised jugular venous pressure with prominent a waves and x descent and bilateral pedal oedema. This was simulating a coloured spectacle. Echocardiographic examination showed evidence of restrictive cardiomyopathy. An abdominal fat pad biopsy stained with Congo red revealed amyloidosis (figure 2). Treatment was started for heart failure and the patient improved with supportive measures.

Periorbital ecchymoses (‘raccoon eye’) is seen in around 11% patients with amyloid light-chain (AL) amyloidosis.1 The characteristic ‘raccoon eyes’ appearance associated with amyloidosis is due to...



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