VA Morning report 6.19.17: Puffy hands syndrome

Case summary: Ivan de Kouchkovsky presented a case with a unique presenting complaint that generated tons of great learning this morning of a 73M with metastatic, castrate-resistant prostate cancer who presented with bilateral upper extremity edema and contractures.
Top pearls: 
1) Bonus pearl from the daily ditty: wall thickness can help you distinguish bulla (<1mm) versus cyst (1-4mm) versus cavity (>4mm) on chest CT.
2) There is a real diagnosis called “puffy hands syndrome”! This usually refers to IVDU-related bilateral hand swelling, however, a host of rheumatologic and other diseases can present with this finding.
3) The instances in which patients with heart failure can have low-normal BNP (~100) are: obesity, isolated R heart failure, and pericardial effusion.
 

Cystic lung disease:
  • Recall that cystic lung diseases are an important cause of pneumothorax
  • Nifty nomenclature based on wall thickness on CT chest
 
Wall thickness
Nomenclature
<1mm
bulla (bleb if at parenchyma surface)
1-4mm
cyst
>4mm
cavity
 

Differential diagnosis of bilateral swollen digits or “puffy hands syndrome”:
  • chronic IVDU (causes sclerosis leading to lymphedema)
  • CPPD
  • gout
  • infectious tenosynovitis
  • reflex sympathetic dystrophy
  • eosinophilic fasciitis
  • palmar fasciitis-polyarthritis
  • palmar fibromatosis
  • HCV arthropathy
  • amyloidosis
  • cryoglobulimemia
  • diabetic cheiroarthropathy
  • RS3PE syndrome (remitting seronegative symmetrical synovitis with pitting edema)
  • early scleroderma
  • rheumatoid arthritis
  • paraneoplatic inflammatory arthritis
  • medication reaction
Note that Stemmer’s sign is a physical exam finding characterized by an inability to pinch or lift the thickened skin fold at the base of an affected digit and is found in lymphedema.
Del Giudice P, Durant J, Dellamonica P. Hand Edema and Acrocyanosis: “Puffy Hand Syndrome”. Arch Dermatol. 2006;142(8):1065-1086.
Alexandroff, BL, et al. Woody hands. Lancet, 361 (2003), p. 1344.

HF + low-normal BNP (~100):
  • obesity
  • isolated R heart failure
  • pericardial effusion
Note that Entresto (sacubitril/valsartan), in contrast, will artificially elevate the BNP, as it prevents cleavage.
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