A 67-year-old Caucasian male was admitted to our hospital due to general fatigue, weight loss and chronic diarrhea. Physical examination revealed a pale, malnourished man with pedal edema and hypotension. Initial laboratory exams showed renal impairment, nephrotic range proteinuria, and cholestatic jaundice. A thorough investigation for secondary causes of nephrotic syndrome was performed including computed tomography imaging, endoscopic evaluation, testing for viral and bacterial infections, and immunological assessment. Echocardiography revealed severe concentric ventricular thickening and diastolic dysfunction along with elevated NT-proBNP levels. Surprisingly, during hospitalization he developed unprovoked bilateral periorbital ecchymoses as well as an extended ecchymosis after abdominal wall fat pad biopsy. Meanwhile, plasma and urine immunofixation revealed monoclonal lambda free light chains with Bence-Jones proteinuria. Biopsies from the petechial mucosa of the ascending colon showed dense amyloid deposits with lambda light chain staining on immunohistochemistry and green birefringence on polarized light microscopy when stained with Congo red dye. On the basis of the above clinical and immunohistological findings we made the diagnosis of systemic light chain amyloidosis (AL). Amyloidosis (AL) is a kaleidoscopic disease manifested with a constellation of nonspecific symptoms such as unexplained proteinuria, orthostatic hypotension, gastrointestinal motility issues, peripheral neuropathy with autonomic features, hepatomegaly, cholestatic jaundice, restrictive myocardiopathy, and bleeding diathesis. The spontaneous appearance of periorbital ecchymoses, also known as raccoon eyes sign, is an uncommon but pathognomonic symptom of amyloidosis attributable to increased vascular fragility from accumulation of amyloid fibrils. Herein, the raccoon eyes sign should prompt internists including amyloidosis in differential diagnosis to prevent diagnostic delays.

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Corresponding author
Nikolaos Sabanis, Department of Nephrology, General Hospital of Trikala, Trikala, Greece (nikospsamanis@yahoo.gr)