Nephrogenic Systemic Fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a condition that --so far -- has occurred only in people with kidney disease. NSF is characterized by hard, woody skin.

NSF/NFD typically starts with swelling and tightening of the skin, frequently with red or dark patches. Usually only arms and/or legs are involved. NSF/NFD can progress rapidly to woody, thickened skin that can become so severe that you cannot walk. There is no consistently effective treatment.

Prior to 1997, there were no Nephrogenic Systemic Fibrosis cases identified. NSF is a systemic disorder which prominently and visibly affects the skin. For this reason, Nephrogenic Fibrosing Dermopathy (NFD) has been suggested as an equivalent terminology.

Neither the duration of kidney disease nor its underlying cause are related to the development of NSF. Some patients with NSF develop skin tightening in the earliest stages of kidney disease while others may have had kidney disease for years.

NSF appears to affect males and females equally. NSF has been confirmed in people of all ages, including children. Ethnic backgrounds do not appear to be a factor.

What are the Nephrogenic Systemic Fibrosis symptoms?
Patients with NSF describe swelling and tightening of the skin, usually limited to the limbs but sometimes involving the trunk. The condition may develop over a period of days to several weeks. In many cases, the skin thickening inhibits extension of joints, resulting in contractures. Severely affected patients may be unable to walk or to fully extend the joints of their arms, hands, legs, and feet. Complaints of muscle weakness are common also.

Changes in the skin may begin as reddened or darkened patches, bumps, or plaques. Then the skin may feel woody (inflexible) and the surface can resemble the texture of an orange peel. Patients may experience burning, itching, or severe sharp pains. Radiography might reveal calcifications of the soft tissue. Deep bone pain can happen in the hips and ribs.

Skin lesions between ankles and thighs are common followed by the wrist and upper arms. Hand and foot swelling with blister-like lesions has been reported. Some patients have reported yellow bumps or plaques on or near the eyes. Rapid or new onset fluctuating hypertension happens prior to the onset of the skin lesions.

What is the treatment for NSF?
There is no consistently successful treatment for NSF, although improving renal function seems to slow or arrest NSF and in many cases allows for gradual reversal of the process over time.

Treatments tried and continue to be investigated include oral steroids (prednisone); topical Dovonex (under occlusion); extracorporeal photopheresis (ECP); plasmapheresis, Cytoxan; Thalidomide; ultraviolet therapy; physical therapy; Pentoxifyllin; high dose Intravenous Ig therapy; and renal transplantation.

What is the prognosis for NSF?
Because NSF is a rare, relatively recent diagnosis, the history of the disease is not well understood. Some patients report a gradual improvement in mobility and slight softening of the skin over time. Complete spontaneous healing in a patient with ongoing kidney disease has not been reported.

Several patients with NSF have died as a result of complications of kidney disease or transplant surgery. One patient, who elected to discontinue dialysis, had widespread fibrosis involving the diaphragm, psoas muscles, proximal esophagus, and intimal areas of vessels of the kidney and lungs.

Less than 5 percent of patients with NSF have an exceedingly rapid and severe disease course that may result in death. NSF, by itself, is not considered fatal, but it can contribute to death by restricting effective ventilation, by restricting mobility to the point of causing an accidental fall that may be further cause fractures and clotting complications, and by other means.

Product Negligent Lawsuit
Are you or a loved one experiencing woody, hard skin because you had kidney problems and had a MRI? Or perhaps you didn't know you had kidney problems and had a MRI? Talk to a law firm knowledgeable about product negligence cases. Let Anapol Schwartz help you.