Calciphylaxis

Overview

Calciphylaxis occurs when calcium deposits build up in the blood vessels, obstructing blood flow to certain skin regions. This disease results in the formation of blood clots, the development of painful skin ulcers, and the potential for severe infections that can be fatal.

In rare instances, calcium deposits may form internally in various organs like the eyes, lungs, brain, muscles, and intestines. When it affects the skin’s surface, calciphylaxis is very painful, causing open wounds similar to bedsores. The skin and surrounding tissue near the wound undergo necrosis, causing the damage from the dead tissue to extend outward. The healing process for these wounds is slow and may lead to significant complications.

Calciphylaxis is typically experienced by individuals who has kidney failure, is undergoing dialysis or has undergone a kidney transplant. This condition can also manifest in individuals without pre-existing kidney disease.

Symptoms

Calciphylaxis typically presents with intense pain, often initiating prior to the manifestation of lesions or visible symptoms. The affected areas can exhibit heightened sensitivity to pressure or touch, further intensifying the overall pain.

Other symptoms include:

• Painful, deeply seated lumps that eventually form open sores covered in dark-brown crusts, persistently resisting healing. These lesions typically manifest in fatty skin areas,such as the abdomen and thigh, although they can develop in any part of the body.
• Skin with large purple net-like patterns.
• Infections resulting from non-healing wounds.
• Vision problems, internal bleeding, and muscle damage.

Causes

Although the precise cause of calciphylaxis is yet unknown, the information that is currently available points to multiple potential causes.

Recent research indicates that individuals with this condition often exhibit abnormalities in blood-clotting factors, which are substances in the blood that aid in preventing excessive bleeding. These irregularities may result in an increased frequency of the formation of small blood clots compared to the usual occurrence.

Other studies suggest that imbalance in the metabolism of calcium can result in calciphylaxis. This results in the deposition of calcium in the arterioles which ultimately causes blood clots to form there. Skin and adipose tissues can become oxygen- and nutrition-deprived due to blood clots.

Risk factors

Calciphylaxis primarily affects individuals in the advanced stages of kidney failure, with potential risk factors including being female, being obese, diabetes, abnormalities in blood-clotting factors, long-term dialysis, and, at times, kidney transplantation.

Other risk factors include:

• An imbalance in the body’s levels of phosphorus, calcium, and aluminum
• Hyperparathyroidism, a disorder marked by an excess of parathyroid hormone (PTH), which controls the body’s levels of calcium and phosphorus
• Uremia, or a condition in which the body accumulates substances like calcium and phosphorus that are typically excreted in the urine
• Certain drugs, like corticosteroids, calcium-binding agents, or warfarin