Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?

Nephrogenic fibrosing dermopathy (NFD) is an acquired, idiopathic disorder that is observed in patients with renal disease. Most patients with NFD have undergone dialysis for renal failure [1,2]. It tends to affect mostly the middle-aged. An association of NFD with coagulation abnormalities, recent vascular surgery or intervention (e.g. shunt/fistula and angioplasty), and presence of antiphospholipid antibodies has been discussed by several authors thus far [1,3], but the origin of the disease is still unknown. A more widespread variant of this fibrosing skin disease with involvement of other organs (e.g. lungs, liver, muscles and the heart) is described as nephrogenic systemic fibrosis (NSF) by Leboit [4], Ting et al. [5] and Daram et al. [6]. NFD is characterized clinically by thickening, induration and hardening of the skin. Distinct nodules also can be seen. The (distal) extremities are the most common area of involvement, followed by the trunk, and the face is almost never involved [1]. The diagnosis of NFD is confirmed in a skin biopsy by specific histopathologic features, namely thickened collagen bundles with surrounding clefts, mucin deposition and a proliferation of fibroblasts and elastic fibers. Signs of inflammation are absent, which makes this disorder a distinct entity [1,7]. In this report, nine end stage renal disease patients undergoing magnetic resonance (MR) angiography are presented, in five of whom skin changes of nephrogenic fibrosing dermatopathy became apparent about 2–4 weeks after the administration of gadolinium (Gd)-containing contrast agent for MR. Patients with and without NFD were compared for possible risk factors to develop this skin disease. Gd is thought to be safe as a contrast agent in renal failure. This case series however, demonstrates that Gd–DTPA possibly plays a triggering role in the development of NFD under certain circumstances.