A New Disease of the Skin

Occasionally in modern medicine, there are still actually “new” diseases. When a new disease is described, such as AIDS in the 1980’s, it is remarkable how quickly the medical community attacks the problem, learns the cause, and starts to develop treatments. What once took decades, now takes only a few years. For example, the first cases of AIDS were being described in 1981 and by 1987 successful trials of the drug AZT had begun.

A new disease in Dermatology:

In 2000 the first report was published describing a new disease in dermatology and a group of fifteen patients who had it. The disease consisted of a hardening of the skin of the hands and feet in patients who had end-stage renal disease and who were receiving dialysis. The hardening of the skin was progressive in many patients causing loss of mobility of the joints, leaving them in pain and often unable to walk or use their fingers and hands. The cause was not dialysis, because dialysis has been around for decades and this disease, called nephrogenic fibrosing dermopathy (NFD), was not seen until 2000.

In 2005 a registry was created to track patients with this new disease and more than 170 cases had been reported or published. But the cause was still unknown, and there were no effective treatments for this horribly debilitating condition that often ended in death.

A possible breakthrough:

Then in mid-2006, The Danish Health Authority notified the FDA of 25 reported cases of nephrogenic fibrosing dermopathy from two European medical centers. All of these cases occurred in patients with renal failure who underwent MRI and received a single dose of a gadolinium-containing contrast agent. (Contrast agent is a chemical injected into the vein of a patient before undergoing MRI. The contrast highlights certain structures in the body on an MRI scan allowing the radiologist to see organs and other structures more clearly.) All of the patients developed NFD within 3 months after receiving the contrast.

Worldwide, approximately 200 cases of NFD have now been reported. Efforts are underway to determine how many of these patients received gadolinium-containing contrast agents. There is still no cure and gadolinium-contrast is only an “association” and not yet a known “cause” of the disease. However, this new disease illustrates how the medical community of the world collaborates to tackle new diseases. .