Nephrogenic Fibrosing Dermopathy Improved after Renal Transplantation / 대한피부과학회지

ABSTRACT

Nephrogenic fibrosing dermopathy (NFD) is a rare cutaneous fibrosing disorder that primarily affects patients with a history of renal disease. NFD manifests with induration, thickening and hardening of the skin with brawny hyperpigmentation. Lesions are typically symmetrical and usually develop on the limbs and trunk. Flexion contractures of the joints may be a feature of the disease. Histopathological features of NFD include proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers and focal mucin deposition. Although the pathogenesis remains largely unknown, some of the factors implicated in the pathogenesis include renal dysfunction, circulating fibrocytes, vascular injury, and gadolinium which is a contrast material used in magnetic resonance imaging. Currently, no definitive or uniformly effective therapies are available for the treatment of NFD. We herein describe the case of a 44-year-old female NFD patient who undergoes significant improvement of skin lesions and associated joint contracture after renal transplantation.