Nephrogenic Fibrosing Dermopathy: A Case Study
Sherril Sego

S.L. is a 32-year-old patient on hemodialysis who presented to her nephrologists with complaints of worsening pain and stiffness in her hands and arms. She described the feeling as hurting to touch and feeling too stiff to bend her fingers. She had, over the previous few weeks, lost much of her ability to perform her own activities of daily living and was rapidly becoming totally dependent on her family for her personal care. She denied any fever or chills, and had no recent medication changes. She has been on hemodialysis for nearly 2 years due to kidney failure from diabetes. S.L. has had a complex history of poorly controlled type 1 diabetes from the age of 10, two myocardial infarctions, and two unsuccessful revascularization surgeries for lower extremity peripheral vascular disease. In spite of all these health problems, she was a happy, energetic mother and wife.

Intended Outcomes

1. The patient will maintain her functional independence in all activities of daily living.
2. The patient will have an acceptable method of controlling her pain and discomfort.

Case Presentation
S.L.’s vital signs were normal at this office visit. Hercentral venous catheter site for hemodialysis access was clear, without redness or drainage. Her heart and lungs were clear, and she had 2+ edema in both ankles. The laboratory values showed mild anemia and uremia, but were consistent with those of a patient with kidney failure. S.L.’s glycosylated hemoglobin level, however, was consistently above normal at 9.7 mg/dL. She was maintained on insulin, with a regimen of insulin glargine at bedtime and insulin aspart before meals and snacks. She had frequent hypoglycemic episodes, usually due to poor eating habits.

On examination, the skin on S.L’s hands and arms appeared thickened, with scattered plaque-like areas and indurated nodules. No lesions or areas of definite redness were noted. Pulses were palpable, but even light pressure on the skin elicited pain. The skin had a peau d’orange appearance and felt “tough.” Her hands and fingers were slightly mottled and puffy. S.L was unable to grasp the examiner’s fingers, but merely make a “C” with her fingers.

The complete blood count was notable for decreased hemoglobin and hematocrit. The Westergren sedimentation rate was elevated at 50 mm/hr. Rheumatoid factor, anti-nuclear antibody, anti-cardiolipin antibody, complement fixation, and serum protein electrophoresis were all within normal limits.

After several consultations for laboratory work and follow- up visits for those reports, a dermatology appointment was scheduled and a skin biopsy was done at that time. The biopsy revealed a diffuse eosinophilic infiltration of the entire dermis. After consultation with the Mayo Clinic, the diagnosis of nephrogenic fibrosing dermopathy (NFD) was made.

Discussion
The exact cause of NFD is unknown (Cowper, 2007). NFD is also known as nephrogenic systemic fibrosis as it may extend to connective tissue and extensive fibrosis of other organs, with the potential to be a fatal disease (Food and Drug Administration [FDA], 2007a). There is no known genetic or gender component. Definitive diagnosis of NFD is confirmed by skin biopsy (FDA, 2006; 2007a).

The majority of patients diagnosed with NFD have undergone hemodialysis for renal replacement therapy. It has also occurred in patients with moderate to end stage renal disease (FDA, 2007a; 2007b). A connection among renal failure, hemodialysis, and NFD continues to be suspected, but other factors such as beta-2 microglobulin accumulation, amyloid deposition, and reaction to artificial dialyzer membranes have been postulated (though not yet substantiated) to be involved in the development of this disease.

A 2006 FDA advisory indicated a possible connection between NFD and the use of gadolinium, a radioactive contrast agent used in magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) (FDA, 2006). As of December 2006, the FDA had received reports of 90 patients with moderate to end stage kidney disease who had developed NFD after having MRI/MRA imaging studies using a gadolinium-based contrast medium (FDA, 2007c).

NFD is characterized by induration, thickening, and hardening of the skin with peau d’orange and brawny hyperpigmentation. The skin is often shiny and feels hard to the touch with a woody consistency. The extremities are the most common areas of involvement, followed by the trunk. The face is almost never involved. Severe pain and joint contractures are common (Cowper, 2007).

Primary nursing interventions for S.L. were aimed at pain management, patient education, and physical therapy to preserve and improve joint function. Pain management became extremely difficult as the disease progressed rapidly and joint contractures developed. Neither oral nor transdermal analgesic medications adequately managed her pain. Physical therapy proved too painful to endure, and her contractures progressed until she was basically unable to use her hands and arms. S.L. became confined to a scooter and required extensive occupational therapy to assist her with simple tasks such as feeding herself.

Cowper, S.E. (2007). The international center for nephrogenic fibrosing dermopathy research. Retrieved November 18, 2007, from http://www.icnfdr.org
 
Food and Drug Administration (FDA). (2006). Public health advisory: Gadolinium-containing contrast agents for magnetic resonance imaging (MRI): Omniscan, OptiMARK, Magnevist, ProHance, and MultiHance. Retrieved November 18, 2007, from http://www.fda.gov/cder/drug/advisory/gadolinum_ agents.htm
 
Food and Drug Administration (FDA). (2007a). Gadolinium-based contrast agents for magnetic resonance imaging (MRI) (marketed as Magnevist, MultiHance, Omniscan, OptiMARK, and ProHance). Retrieved November 18, 2007, from http://www.fda.gov/cder/drug/InfoSheets/HCP/gcca_200 705HCP.pdf

Food and Drug Administration (FDA). (2007b). Update on magnetic resonance imaging(MRI) contrast agents containing gadolidium and nephrogenic systemic fibrosis. Retrieved November 18, 2007, from http://www.fda.gov/cder/drug/ advisory/gadolinium_agents_20061222.htm

Food and Drug Administration (FDA). (2007c). Update on magnetic resonance imaging (MRI) contrast agents containing gadolinium and nephrogenic fibrosing dermopathy. Retrieved November 23, 2007, from http://www.fda.gov/cder/drug/advisory/ gadolinium_agents_20061222.htm