The Journal of the Arkansas Medical Society Med Journal June 2020 | Page 17
derm dilemma
Special Section: Short Dermatological Cases
by Amanda ederle, md candidate, class of 2020
uams dept of dermatology
A 67-year-old female with medical history significant
for end stage renal disease, diabetes mellitus,
and morbid obesity presents to the emergency
room with a five-to-six-day history of livedo
reticularis and exquisitely painful, firm, stellate
purpura of the thighs, lower legs, abdomen, and
buttocks. The patient also has a history of secondary
hyperparathyroidism and an elevated calcium-phosphate
product. Physical examination
revealed the findings seen in the image..
The therapeutic intervention most likely to result
in long-term control and decreased morbidity/
mortality is:
A. Irrigation and vigorous debridement
B. Intravenous sodium thiosulfate
C. Reassurance and observation
D. Peritoneal hemodialysis
E. Intravenous vitamin K
Answer: B - IV sodium thiosulfate
The patient has calciphylaxis, a relatively uncommon
disorder occurring primarily in the setting of
end stage renal disease, which is characterized by
painful ulcerations and necrosis of the skin and
subcutaneous tissue. Mortality is high, and often
the result of sepsis. Though the pathogenesis
of calciphylaxis is not fully understood, clinical
findings suggest it results from ischemia and
necrosis caused by calcification and subsequent
thrombus formation within arterioles of the skin
and subcutis. Risk factors for calciphylaxis include
end stage renal disease, female sex, obesity,
hypercalcemia, hyperphosphatemia, elevated
parathormone level, and concurrent use of warfarin.
Areas of the body with high fat content, such
as the lower extremities, abdomen, and buttocks,
are typically affected. Deep-wedge biopsy demonstrates
calcification within the media of small- and
medium-sized arterioles (which may be visualized
with plain radiography), intimal hyperplasia and
fibrosis, and intraluminal microthrombi. In addition
to the painful skin lesions, some patients will
have concurrently elevated calcium, phosphorus,
or parathyroid hormone; thus, phosphate binders,
low calcium bath dialysis, bisphosphonates,
calcimimetics, and even subtotal parathyroidectomy
may be employed. The six-month mortality
rate of this disorder is approximately 50%, and
mounting evidence suggests that IV sodium
thiosulfate, a potent antioxidant, is effective in
solubilizing calcium. Meticulous wound care,
pain management, and treatment of secondary
microbial infections are also important aspects of
treatment. Sodium thiosulfate therapy is typically
continued for several months, and response is
monitored by a decrease in the number, size, or
pain of calciphylaxis ulcerations/eschars.
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Volume 116 • Number 12 JUNE 2020 • 281