Description of 12 Cases of Nephrogenic Fibrosing Dermopathy and Review of the Literature

https://doi.org/10.1016/j.semarthrit.2005.08.002Get rights and content

Objectives

To review the clinical and laboratory features of 12 cases of nephrogenic fibrosing dermopathy (NFD) studied at our institution and of 70 previously described cases in the literature.

Methods

Clinical evaluation and laboratory studies of 12 patients with NFD associated with chronic hemodialysis or peritoneal dialysis for end-stage renal disease and a review of 23 previous publications describing 70 patients with this disease.

Results

Eleven patients undergoing chronic hemodialysis and 1 patient undergoing chronic peritoneal dialysis for end-stage renal failure developed a severe and progressive cutaneous fibrotic process with woody induration of legs, thighs, hands, and forearms, and severe loss of motion and flexion contractures in multiple joints. Several patients displayed systemic involvement including fibrosis of muscles, myocardium, and lungs and marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein. Three patients died within 2 years of symptom onset. A review of previously published reports of this disorder confirmed the presence of systemic involvement and a poor prognosis with a high mortality rate.

Conclusions

NFD is a severe and usually progressive systemic fibrotic disease affecting the dermis, subcutaneous fascia, and striated muscles. It also appears that the disease can cause fibrosis of lungs, myocardium, and other organs.

Section snippets

Methods

Clinical, laboratory, and histopathological studies were performed in 10 patients evaluated at the Scleroderma Center of Thomas Jefferson University between the years 2000 and 2005, in 1 patient followed at the Veterans Administration Hospital of St. Louis, MO, and in 1 patient followed at the Robert Wood Johnson Medical School (Camden, NJ). This evaluation included a detailed assessment for the presence of systemic involvement, including a review of systems and physical examination for

Demographical and Epidemiological Characteristics

The details of the epidemiologic and demographic data of the 12 patients we studied are shown in Table 1. Ten patients received hemodialysis (HD), 1 patient received peritoneal dialysis (PD), and another patient was on HD at the time of diagnosis but had received PD previously. Six of 12 patients had renal transplantation which failed in all and required removal. The cause of the renal failure was diverse, as well as the duration of dialysis (Table 1).

Clinical Features

The clinical features of the 12 patients we

Discussion

NFD is a severe, usually progressive, fibrotic disorder occurring in patients with end-stage renal disease who received, with few exceptions, hemodialysis or peritoneal dialysis. In addition to the severe cutaneous alterations, frequently there is evidence of systemic involvement including: (1) a very marked elevation of the erythrocyte sedimentation rate and C-reactive protein; (2) tendinous, peri-articular, and striated muscle involvement; and (3) visceral fibrosis of heart, lungs, and other

Acknowledgments

This work was supported by NIH Grant AR19161 (to S.A.J.). Dr. Nora Sandorfi was supported by NIH training Grant AR07583. Dr. Sonsoles Piera-Velazquez was supported by a Postdoctoral Fellowship from the Arthritis Foundation. The expert assistance of Kate Salmon in the preparation of this paper is gratefully acknowledged.

References (30)

  • W.W. Ting et al.

    Nephrogenic fibrosing dermopathy with systemic involvement

    Arch Dermatol

    (2003)
  • S.A. Jimenez et al.

    Dialysis-Associated Systemic Fibrosis (Nephrogenic Fibrosing Dermopathy)

    Arthritis Rheum

    (2004)
  • J.M. Levine et al.

    Involvement of skeletal muscle in dialysis-associated fibrosis (Nephrogenic Fibrosing Dermopathy)

    Muscle Nerve

    (2004)
  • K. Hershko et al.

    A variant of nephrogenic fibrosing dermopathy with osteoclast-like giant cellsa syndrome of dysregulated matrix remodeling?

    J Cutan Pathol

    (2004)
  • H. Chiu et al.

    Nephrogenic fibrosing dermopathya rare entity in patients awaiting liver transplantation

    Liver Transpl

    (2004)
  • Cited by (237)

    • Non-iodinated contrast media nephrotoxicity

      2018, Nephrologie et Therapeutique
    View all citing articles on Scopus

    Supported by NIH Grant AR19616.

    View full text