Nephrogenic Systemic Fibrosis: Mini-Review

The patient was a 43-year-old male who had been diagnosed with Alport syndrome and dialytic chronic kidney disease in 1998. In November 2006, he was admitted to the hospital due to septic shock secondary to a hemodialysis catheter infection that developed into a femoral prosthesis infection. One month later, the patient underwent two MRI scans as preparation for the surgical prosthesis substitution. A gadolinium-based contrast agent was used with a gadodiamide dose of 0.15 mmol/kg in each exam, with a one-week interval between exams. Two months after the MRI, the patient had thickening and hardening of the skin of the right forearm (Figure 1) that extended to the remaining limbs, as well as to a band lesion in the left lower limb (Figure 2). The remaining physical examination was normal. The laboratory findings included an increase in C-reactive protein to 52 mg/L, with a slight increase in serum ferritin to 320 ng/mL. Other tests were normal, such as the hemosedimentation velocity and serum albumin concentration. The pulmonary function test and the echocardiogram were both normal. A computed tomography (CT) scan of the affected areas showed diffuse thickening of the muscle, skin and subcutaneous tissue. The patient then underwent muscle and skin biopsies. Nephrogenic systemic fibrosis (NSF) was confirmed in the skin and muscle biopsies. Immunohistochemical analysis showed the presence of CD34+, CD68+ and factor XIIIa+ Figure 1 Thickening and hardening of the skin of the right forearm