Nephrogenic fibrosing dermopathy (
NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. It appears similar to
scleromyxedema but with some notable exceptions, including the lack of involvement of the face and absence of plasma cells on histology, systemic involvement, and
paraproteinemia. Patients can present with thickened or edematous skin with indurated papules and plaques involving the extremities and the trunk. We report the first three cases of
NFD after
liver transplantation successfully treated with
plasmapheresis. Two patients underwent
liver transplantation for hepatitis C virus-induced
cirrhosis and one for hepatitis B virus-induced
cirrhosis. All the patients had
encephalopathy, refractory
ascites, and
malnutrition prior to
transplantation. Like those patients with
NFD, all three of our patients had renal dysfunction and required
hemodialysis before and after
transplantation. Two were not dependent on dialysis at the time of diagnosis, however. These patients had excellent liver allograft function, but the other patient had allograft failure secondary to recurrent
hepatitis C. Immunosuppression therapy consisted of
basiliximab,
mycophenolate mofetil,
calcineurin inhibitor, and
prednisone. The patients developed "woody" skin induration of the distal extremities, erythematous papules, and
contractures at 1, 2, and 120 months after
transplantation. Skin biopsies resembled
NFD. No
paraproteinemia was evident. One to three 5-day courses of
plasmapheresis resulted in moderate to marked clinical improvement. The improvement of the kidney function in two of our patients did not appear to correlate with that of the skin disorder, because the kidney function was improving at the time the diagnosis of
NFD was made. In conclusion, we report the first three cases of
NFD after
liver transplantation.
Plasmapheresis was moderately successful in resolving the skin-indurated papules, severe skin induration, and associated joint
contractures. Preliminary studies (unpublished data) show that decreasing plasma levels of
transforming growth factor-beta1 after
plasmapheresis appear to correlate with the amelioration of this clinical condition.