The nailfold dermoscopy findings of patients with atypical haemolytic uremic syndrome

Abstract

Hemolytic uremic syndrome (HUS) is a severe disease characterized by microangiopathic anemia, thrombocytopenia, and acute renal failure. Atypical HUS (aHUS) that results due to genetic disorders of the alternative complement pathway results in inflammation, endothelial damage, and kidney injury. Therefore, simple and non-invasive tests are needed to evaluate the activity of the disease by assessing the microvascular structure in aHUS. A dermoscope (×10) is an inexpensive and easily portable device used to visualize nailfold capillaries and has high clinical performance and interobserver reliability. In this study, the nailfold capillaries of patients with aHUS who were in remission under eculizumab treatment were examined, and the findings were compared to those of a healthy control group to evaluate disease characteristics. All children with aHUS had decreased capillary densities even if they were in remission. This may be indicative of ongoing inflammation and microvascular damage in aHUS. A dermoscopy can be used as a screening tool for disease activity in patients with aHUS.