New skin disorder seen in renal disease

Nephrogenic fibrosing dermo-pathy is a newly identified skin disorder that appears to be strongly associated with renal disease, dialysis, and renal transplantation. First described in 2000 by one doctor of Yale University, New Haven, and his associates, the scleroderma look-alike condition has now been identified in 70 patients across the United States and Europe, reported by doctors of California Department of Health Services. Kidney disease is a common link in all patients but one, and in that case, the patient may have had transient renal failure, as reported at the annual meeting of the Pacific Northwest Dermatological Society. Professor and Chairman of Dermatology at the University of Arkansas, Little Rock, reviewed important points about the disease at the meeting.

He presented the case of a 56-year-old woman with end-stage renal disease who was undergoing dialysis. She complained that the skin on her legs had become progressively thickened, scarlike, and tight, although she had sustained no trauma to the region. Her arms were affected in a similar manner but to a lesser degree. Over time, she had developed contractures and erosions that eventually confined her to a wheelchair. For all the world, it looked scleroderma. If you bounced a nickel on the skin of her legs, it would bounce across the room. The skin was that tight. An incisional biopsy revealed dense nodules of fibroblasts in the reticular dermis, with a marked mucin accumulation. The histology differed from what would be seen in a scleroderma patient, where all the interstices disappear and collagen bundles expand to basically push everything aside.

Scleromyxedema can produce a similar fibroblastic picture, but again, there is a key differentiation since patients with nephrogenic fibrosing dermopathy have no associated IgG- paraprotein. A number of treatment approaches, including prednisone with or without plasmapheresis, have been tried. No strategy has been consistently successful. When nephrogenic fibrosing dermopathy was originally identified in eight of 265 kidney transplant recipients at a single Southern California transplant center, it seemed likely to be due to some infectious entity at that site. But reports soon began to trickle in the suggest a more ubiquitous exposure by kidney disease patients to contamination or some infectious process. About half of the reported cases are in renal transplant patients, but many other people with classic symptoms and hispathology have only undergone dialysis or been treated for renal disease.

A very small case-control study among the original cluster of patients showed that those with nephrogenic fibrosing dermopathy were more likely than others to have had less than optimal renal function after transplantation, requiring hemodialysis and medications associated with severe disease. A similar case has presented with large areas of painful skin on her arms and legs that had development over the prior six months. A skin biopsy showed changes suggestive of scleromyxedema. A colloidal iron stain was positive for mucin. A CD34 stain was positive. The patient had neither monoclonal gammopathy nor paraproteinemia. My theory is that something related to the process of dialysis had changed over the last two years. People with end-stage renal disease used to be dialyzed in 5-8 hours. Now they’re dialyzed for three hours using new machines with different filters that can speed up the process. My sense is that something is leeching from these plastic filters or in the dialysate to cause this skin disease.