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Calciphylaxis
See also in: Cellulitis DDx
Other Resources UpToDate PubMed

Calciphylaxis

See also in: Cellulitis DDx
Contributors: Amy E. Blum MA, Philip I. Song MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Calciphylaxis, also known as calcific uremic arteriolopathy, is a microvascular occlusion syndrome thought to be due to diffuse deposition of insoluble calcium salts in cutaneous blood vessels with associated thrombosis. While the exact pathogenesis is unclear, characteristic pathologic findings include progressive medial calcification of cutaneous blood vessels and subsequent ischemic necrosis of the skin. The process may be triggered by chronic hypocalcemia from decreased intestinal absorption of calcium, leading to increased levels of parathyroid hormone (PTH) and subsequent recruitment of calcium and phosphate from bone. Hypercoagulable states are also thought to play a possible role.

Calciphylaxis is increasing in incidence and is most commonly associated with chronic renal failure, hemodialysis, and secondary hyperparathyroidism. There are also many cases of "nonuremic" or "nontraditional" calciphylaxis, which can occur in the setting of liver disease, diabetes, warfarin use, use of calcium-based phosphate binders, systemic corticosteroid use, solid organ malignancies, systemic lupus erythematosus, and Crohn disease. Other risk factors include female sex, obesity, Northern European descent, and hypoalbuminemia.

Notably, warfarin-associated nonuremic calciphylaxis tends to occur about 2.5 years after warfarin initiation on the lower extremities, does not have associated calcium abnormalities, and appears to have a more favorable prognosis than calciphylaxis associated with renal failure states.

Early lesions are extremely painful, violaceous retiform patches and plaques, classically on fat-bearing areas such as the thighs, buttocks, or abdomen. This is followed by necrosis, ulcers, eschar formation, and possibly gangrene. Induration of the surrounding tissues may be present. Lesions have been reported to be triggered by local trauma, including from insulin or heparin injections, or a skin biopsy. Most lesions develop over the course of weeks to months, while some may progress more rapidly.

Mortality from calciphylaxis is high (60%-87%) and is largely secondary to sepsis from large, nonhealing ulcers.

Codes

ICD10CM:
E83.59 – Other disorders of calcium metabolism

SNOMEDCT:
237900002 – Calciphylaxis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:04/14/2020
Last Updated:05/06/2020
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Calciphylaxis
See also in: Cellulitis DDx
Calciphylaxis : Buttocks, Painful skin lesions, Eschars, Arms, Legs, Skin ulcers, Retiform purpura, warfarin
Clinical image of Calciphylaxis
Circumferential, erythematous plaque on the lower leg.
Copyright © 2021 VisualDx®. All rights reserved.