Pathognomonic STEW: visible clues to systemic amyloidosis Herbert L. Fred, MD, and Hendrik A. van Dijk
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ystemic amyloidosis can affect multiple body parts and can manifest itself in a variety of ways. When present, any of the clues shown in the Figure can facilitate the diagnosis, minimizing unnecessary testing and inappropriate treatment. SHOULDERS • Resemble a football player wearing shoulder pads • Markedly swollen and rubbery hard but not tender • Surrounding structures, especially the muscles, may also be affected • Joint juice is noninflammatory and may contain amyloid fragments or Bence-Jones protein • Other joints may swell concomitantly • Often mistaken for rheumatoid arthritis
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b
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Figure. Signs of systemic amyloidosis involving the (a) shoulders, (b) tongue, (c) eyes, and (d) wrists.
TONGUE • Symmetrically enlarged, often with serrations • Typically thick and smooth, but sometimes nodular • Hemorrhages occasionally dot the surface • Speech may be impaired, providing an audible clue as well EYES • Palpebral and periorbital hemorrhages simulate “raccoon eyes” • Hemorrhages occur spontaneously or after minimal trauma • Waxy, flesh-colored, papulonodular lesions on or near eyelids • Easily induced intralesional bleeding WRISTS • Striking synovial swelling • Thickened tendons over dorsum of hands 372
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Wasting of dorsal interossei Stiffness but no pain or signs of inflammation Subcutaneous nodules common Often mistaken for rheumatoid arthritis
TAKE-HOME MESSAGE Each of these four signs is characteristic of amyloidosis. Any combination of two or more is pathognomonic.
From the Departments of Medicine (Fred) and Graphic Communications (van Dijk), McGovern Medical School, UT Health, Houston, Texas. Corresponding author: Herbert L. Fred, MD, MACP, Emeritus Professor of Medicine, McGovern Medical School, Houston, Texas (e-mail: hlf1929@yahoo. com). Proc (Bayl Univ Med Cent) 2017;30(3):372
S
ystemic amyloidosis can affect multiple body parts and can manifest itself in a variety of ways. When present, any of the clues shown in the Figure can facilitate the diagnosis, minimizing unnecessary testing and inappropriate treatment. SHOULDERS • Resemble a football player wearing shoulder pads • Markedly swollen and rubbery hard but not tender • Surrounding structures, especially the muscles, may also be affected • Joint juice is noninflammatory and may contain amyloid fragments or Bence-Jones protein • Other joints may swell concomitantly • Often mistaken for rheumatoid arthritis
a
b
c
d
Figure. Signs of systemic amyloidosis involving the (a) shoulders, (b) tongue, (c) eyes, and (d) wrists.
TONGUE • Symmetrically enlarged, often with serrations • Typically thick and smooth, but sometimes nodular • Hemorrhages occasionally dot the surface • Speech may be impaired, providing an audible clue as well EYES • Palpebral and periorbital hemorrhages simulate “raccoon eyes” • Hemorrhages occur spontaneously or after minimal trauma • Waxy, flesh-colored, papulonodular lesions on or near eyelids • Easily induced intralesional bleeding WRISTS • Striking synovial swelling • Thickened tendons over dorsum of hands 372
• • • •
Wasting of dorsal interossei Stiffness but no pain or signs of inflammation Subcutaneous nodules common Often mistaken for rheumatoid arthritis
TAKE-HOME MESSAGE Each of these four signs is characteristic of amyloidosis. Any combination of two or more is pathognomonic.
From the Departments of Medicine (Fred) and Graphic Communications (van Dijk), McGovern Medical School, UT Health, Houston, Texas. Corresponding author: Herbert L. Fred, MD, MACP, Emeritus Professor of Medicine, McGovern Medical School, Houston, Texas (e-mail: hlf1929@yahoo. com). Proc (Bayl Univ Med Cent) 2017;30(3):372
