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JAMA Dermatology Patient Page
May 22, 2019

Do I Have Calciphylaxis?

JAMA Dermatol. Published online May 22, 2019. doi:10.1001/jamadermatol.2019.0458

Calciphylaxis is a rare disease that results from calcium clotting small blood vessels of the skin and fat.

The calcium clots in calciphylaxis block blood flow and oxygen delivery to the skin. As a result, the skin becomes very painful and dies, causing wounds with firm black scabs. Calciphylaxis most commonly occurs in those with severe kidney disease, or those taking the blood thinner warfarin. It can also occur if the parathyroid gland is too active, and it has been seen with some cancers. Calciphylaxis is more common in women and in patients who are obese, have diabetes or liver disease, or are taking a steroid medication.

Symptoms

The most striking symptom of calciphylaxis is extreme pain that may be difficult to control despite a high dose of pain killers. The eruption starts with a faint purple lace-like rash. It typically involves the lower legs, thighs, hips, and abdomen but may not involve all of these areas. Over time, the skin develops wounds with large, firm, black scabs. These wounds are slow to heal and may develop infection, which may spread to the blood.

Diagnosis

Your doctor will discuss with you the necessary steps to confirm the diagnosis. These steps include having your skin examined and undergoing blood tests, skin biopsies, and possibly x-rays or other types of imaging. The diagnosis may be difficult to make because the pieces of calcium are so small and scattered that they may be missed in a skin biopsy. Sometimes multiple biopsies are necessary to confirm the diagnosis.

Treatment

Calciphylaxis is a serious, sometimes life-threatening skin disease that is difficult to treat. A team of health care professionals including kidney, skin, surgical, and pain specialists will coordinate your care. A drug called sodium thiosulfate is sometimes injected directly into the blood, and this may gradually halt the disease process, reducing pain and preventing new ulcers from forming. Wound care to prevent infection and pain control are also very important. Other treatments, such as medications to regulate blood levels of calcium and phosphate, and special oxygen treatment in a chamber for the wounds, may be used. Your doctor will also ask you to stop taking any medications that could worsen calciphylaxis, including warfarin, systemic steroids, vitamin D, iron, and calcium.

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Section Editor: Courtney Schadt, MD
The JAMA Dermatology Patient Page is a public service of JAMA Dermatology. The information and recommendations appearing on this page are appropriate in most instances, but they are not a substitute for medical diagnosis. For specific information concerning your personal medical condition, JAMA Dermatology suggests that you consult your physician. This page may be photocopied noncommercially by physicians and other health care professionals to share with patients. To purchase bulk reprints, call (312) 464-0776.
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Article Information

Published Online: May 22, 2019. doi:10.1001/jamadermatol.2019.0458

Conflict of Interest Disclosures: None reported.

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