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Nephrogenic fibrosing dermopathy in a peritoneal dialysis patient
nephrology image
http://www.kidney-international.org & 2007 International Society of Nephrology
Kidney International (2007) 72, 1294; doi:10.1038/sj.ki.5002460
Nephrogenic fibrosing dermopathy in a peritoneal dialysis patient C-W Tsai1,2, C-C Chao3, V-C Wu1, C-H Hsiao4 and Y-M Chen1 1
Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; 2Department of Internal Medicine, Far Eastern Memorial Hospital, Taipei, Taiwan; 3Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan and 4Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan Correspondence: Y-M Chen, Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, No. 7 ChungShan South Road, Taipei 100, Taiwan. E-mail: [email protected]
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Figure 1 | Nephrogenic fibrosing dermopathy involving bilateral lower extremities. (a) Leg stiffness and nearly 901 flexion contractures of the knee. (b) Thickened and waxy skin with hyperpigmentation.
Figure 2 | Skin biopsy from left leg. (a) Orcein stain of the skin showing increased numbers of elastic fibers (original magnification, 200). (b) Immunohistochemistry identifying some interstitial cells in the dermis positive for CD68 (original magnification, 100). CD68 þ or factor XIIIa þ dendritic cells are a constant finding in early stage of nephrogenic-fibrosing dermopathy.
A 69-year-old woman with end-stage renal disease on peritoneal dialysis for 5 years presented with lower extremity weakness of 1-year duration. Transitional cell carcinoma involving the right renal pelvis and ureter was found 2 years after initiation of dialysis and she subsequently underwent bilateral nephroureterectomy. Between 2003 and 2005, the patient received four gadolinium-enhanced magnetic resonance imaging examinations for follow up of the transitional cell carcinoma. However, progressive stiffness with decreased range of motion and flexion contractures of lower extremities developed in the year before presentation. On examination, the skin of her calves and thighs was thickened and waxy with hyperpigmentation (Figure 1). Laboratory studies did not show paraproteinemia or elevation of serum creatine kinase. Both anti-Scl-70 and anti-CENP antibodies were negative. Skin biopsy revealed diffuse fibrosis extending from the
dermis, through subcutaneous fat, and down to the muscles, and immunohistochemical studies revealed interstitial cells positive for CD34, CD68 and factor XIIIa (dermal dendrocytes). These features were compatible with nephrogenicfibrosing dermopathy (Figure 2). Six months after the diagnosis of nephrogenic-fibrosing dermopathy, the patient was admitted with peritonitis and septic shock and later succumbed as a result of nosocomial infection. Nephrogenicfibrosing dermopathy is a scleroderma-like disease exclusively affecting patients with renal failure, and manifests with progressive cutaneous and sometimes systemic fibrosis.
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ACKNOWLEDGMENTS
This report was financially supported by the Ta-Tung Kidney Foundation.
Kidney International (2007) 72, 1294
http://www.kidney-international.org & 2007 International Society of Nephrology
Kidney International (2007) 72, 1294; doi:10.1038/sj.ki.5002460
Nephrogenic fibrosing dermopathy in a peritoneal dialysis patient C-W Tsai1,2, C-C Chao3, V-C Wu1, C-H Hsiao4 and Y-M Chen1 1
Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; 2Department of Internal Medicine, Far Eastern Memorial Hospital, Taipei, Taiwan; 3Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan and 4Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan Correspondence: Y-M Chen, Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, No. 7 ChungShan South Road, Taipei 100, Taiwan. E-mail: [email protected]
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Figure 1 | Nephrogenic fibrosing dermopathy involving bilateral lower extremities. (a) Leg stiffness and nearly 901 flexion contractures of the knee. (b) Thickened and waxy skin with hyperpigmentation.
Figure 2 | Skin biopsy from left leg. (a) Orcein stain of the skin showing increased numbers of elastic fibers (original magnification, 200). (b) Immunohistochemistry identifying some interstitial cells in the dermis positive for CD68 (original magnification, 100). CD68 þ or factor XIIIa þ dendritic cells are a constant finding in early stage of nephrogenic-fibrosing dermopathy.
A 69-year-old woman with end-stage renal disease on peritoneal dialysis for 5 years presented with lower extremity weakness of 1-year duration. Transitional cell carcinoma involving the right renal pelvis and ureter was found 2 years after initiation of dialysis and she subsequently underwent bilateral nephroureterectomy. Between 2003 and 2005, the patient received four gadolinium-enhanced magnetic resonance imaging examinations for follow up of the transitional cell carcinoma. However, progressive stiffness with decreased range of motion and flexion contractures of lower extremities developed in the year before presentation. On examination, the skin of her calves and thighs was thickened and waxy with hyperpigmentation (Figure 1). Laboratory studies did not show paraproteinemia or elevation of serum creatine kinase. Both anti-Scl-70 and anti-CENP antibodies were negative. Skin biopsy revealed diffuse fibrosis extending from the
dermis, through subcutaneous fat, and down to the muscles, and immunohistochemical studies revealed interstitial cells positive for CD34, CD68 and factor XIIIa (dermal dendrocytes). These features were compatible with nephrogenicfibrosing dermopathy (Figure 2). Six months after the diagnosis of nephrogenic-fibrosing dermopathy, the patient was admitted with peritonitis and septic shock and later succumbed as a result of nosocomial infection. Nephrogenicfibrosing dermopathy is a scleroderma-like disease exclusively affecting patients with renal failure, and manifests with progressive cutaneous and sometimes systemic fibrosis.
1294
ACKNOWLEDGMENTS
This report was financially supported by the Ta-Tung Kidney Foundation.
Kidney International (2007) 72, 1294