Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). These disorders have very diverse etiologies and often an unclear pathogenetic mechanism. Distinct clinical characteristics, skin histology, and disease associations may allow one to distinguish these conditions from scleroderma and from each other. A prompt diagnosis is...

Osseous metaplasia has recently been described in several cases of nephrogenic systemic fibrosis, sometimes in association with unusual clinical features such as painful hyperkeratotic spicules, palpable bony masses, and disease regression. Some authors have suggested that it may mainly occur late in the disease course or even be a marker for involuting nephrogenic systemic fibrosis. Here, we p...

Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. Thickening of the skin is typical, usually affecting the extremities. Visceral organs can also be affected. The diagnosis of the disease requires careful clinicopathological correlation. Trea...

Nephrogenic remnants (NRs) are nodular collections of undifferentiated renal blastema cells in the postnatal kidney that are recognized as putative precursor lesions of Wilms tumor (WT). NRs may remain stationary, undergo regression, or proliferate. In the last case, there is a high risk for the development of a WT. During infancy, they are most frequently of microscopic size, to be found only ...

Nephrogenic systemic fibrosis (NSF) is a serious, sometimes fatal disease. Findings in recent years have shown that a causal association between gadolinium containing contrast media and NSF is most likely. Therefore, the regulatory authorities have issued guidelines on the use of gadolinium-containing contrast media which have reduced the number of new cases of NSF to almost zero. However, it i...

We present a patient with progressive dural calcifications, thickening, and enhancement presumably related to the development of nephrogenic systemic fibrosis (NSF). Head CT demonstrated progressive dural calcifications, whereas MR imaging demonstrated progressive dural thickening and enhancement during a 3-year period in which the patient received several gadolinium-enhanced MR imaging studies...