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Images in Dermatology
January 16, 2019

Systemic Amyloidosis

Author Affiliations
  • 1Division of Dermatology, University of Vermont Medical Center, Burlington
  • 2Department of Dermatology and Venereology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University
  • 3Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington
JAMA Dermatol. 2019;155(3):371. doi:10.1001/jamadermatol.2018.4413

An elderly woman presented with swelling of her hands accompanied by burning sensation in her palms of about 2 months’ duration. In addition she described an intermittent “bruise-like” rash on her hands. Of note, her medical history included a recent presumed diagnosis of polymyalgia rheumatica, which was made based on complaints of weakness, intermittent paresthesias, myalgias, and arthralgias. Examination of the hands revealed moderate edema bilaterally with pitting and an overall waxy appearance of the palms. Nonblanchable purple macules were noted on both the palmar (Figure) and dorsal surfaces of the hands. She was unable to completely open or close either hand. There were no other relevant cutaneous findings. A punch biopsy of the left hand demonstrated pink, amorphous, homogeneous hyaline material within the reticular dermis and within the walls of vessels. Congo red histochemical staining was positive, exhibiting green birefringence under polarized light, consistent with amyloid. Initial blood work revealed an erythrocyte sedimentation rate of 81 mm/h, and no anemia or hypercalcemia were noted. Further workup included a positive result from serum protein electrophoresis, positive urine protein electrophoresis, and a bone-marrow biopsy consistent with multiple myeloma.