Selected Skin Diseases With Systemic Involvement

Marcelo Ruiz, MD; Pilar Valdés, MD; Kenneth Tomecki, MD

Disclosures

Skin Therapy Letter. 2013;18(4) 

In This Article

Nephrogenic Systemic Fibrosis in Renal Insufficiency

Nephrogenic systemic fibrosis (NSF), or formerly known as nephrogenic fibrosing dermopathy, is a scleroderma-like fibrosing disorder that develops in the setting of renal insufficiency. It occurs predominately in patients with end-stage renal disease on dialysis (80%) and occasionally in patients with acute renal failure or after kidney transplantation.[6] The cause remains unclear, but most cases are related to the use of gadolinium-based contrast agents (GBCA) in magnetic resonance imaging (MRI), such as gadodiamide and gadopentetate. These gadolinium (Gd) chelates tend to easily release free Gd into the surrounding tissue.[7] In patients with renal insufficiency, increased retention of GBCA leads to increased free Gd that could ultimately trigger NSF.[6] A concomitant proinflammatory event (e.g., infection and major surgery) can confer additional risk.[8]

Cutaneous lesions of NSF usually develop over a variable period of time (few days to several months) and subsequently assume a chronic, unremitting course. Approximately 5% of patients experience NSF that is rapidly progressive (fulminant).[6] The primary skin lesions are usually papules or nodules that are erythematous and coalesce to form indurated irregular plaques. These areas may appear slightly edematous with peau d'orange and erythematous surface features, and can be easily confused with cellulitis, lymphangitis or chronic lymphedema.6 They are often symmetrical, commonly involving the lower extremities up to the knees and the upper extremities up to the elbows. The face is usually spared. Over time, the skin tends to develop a "cobblestone" appearance and browny hyperpigmentation. Affected areas and subcutaneous tissues are extremely hard ("woody"), warm, and tender, as well as often accompanied by pruritus and burning sensation. Yellow scleral plaques are found in some cases.[6,9–11] Differential diagnosis is made with several other fibrosing processes, including scleroderma and scleromyxedema.[11] The clinical distribution of lesions in NFS, which favors the extremities and spars the face, is opposite to that seen in scleromyxedema, and paraproteins are not detected in the serum of NFS patients.[6] Histopathology of NFS is very similar to scleromyxedema, including their immunohistochemical staining profiles. A scoring system, based on clinical and histopathological criteria, has been recently proposed to aid diagnosis.[11]

Major organ or systemic involvement can include muscles, joints (contractures), and viscera, such as the pulmonary, cardiovascular, gastrointestinal and renal systems.[6] The chronology of the visceral involvement is not clear, but is more common in patients exhibiting extensive skin symptoms. The severity and rapidity of progression of the cutaneous lesions correlate with poor prognosis and death.[12]

Therapeutic modalities include oral and topical steroids, pentoxifylline, photopheresis, plasmapheresis, psoralen plus ultraviolet light, IVIG, sodium thiosulfate, and physical therapy, however, all with little benefit. Improvement of renal function can terminate the progression of NSF, so renal transplantation is the best option for these patients. Implementation of preventive strategies, including the use of alternatives to Gd-enhanced MRI, has been shown to reduce the risk of NSF.[6,8–10]

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