Raccoon sign



Illustration 1.

The patient, a 72-year-old man, presented with an 18-month history of fatigue, dizziness, and exercise dyspnea. On physical examination, periorbital ecchymosis (“raccoon sign” or “panda eyes”) and postural hypotension without increased heart rate were noted. Proteinuria was measured by 24-hour urine collection (protein level 2.6 grams), and serum monoclonal paraprotein (κ light chain) was detected. An echocardiogram showed thickening of the ventricular walls and diastolic dysfunction. A bone marrow biopsy specimen contained 46% plasma cells. Soon after diagnosis, he developed pneumonia and died 20 days later. Skin involvement is one of the most characteristic manifestations of primary amyloidosis (AL amyloidosis). Usually nonpruritic lesions consist of slightly raised, waxy papules or plaques clustered in the folds of the axillae, anal, or inguinal regions. Although it occurs only in about one-fifth of patients with AL amyloidosis, periorbital ecchymoses are considered pathognomonic of this disease. These typical lesions are due to intracutaneous hemorrhage, resulting from amyloid infiltration and weakening blood-vessel walls.