Nephrogenic fibrosing dermopathy, a new mimicker of systemic sclerosis

Nephrogenic fibrosing dermopathy is a unique fibrosing disorder recently identified to occur exclusively among patients with renal disease. The cutaneous findings are similar to those of systemic sclerosis, but it is important to differentiate between these two disorders because of significant prognostic and therapeutic implications. Nephrogenic fibrosing dermopathy is usually a diagnosis of exclusion, but the condition does have distinct clinical and histopathologic findings. It appears to be multifactorial in pathogenesis, and no specific cause has been identified. No specific treatment modality has been consistently effective, but there have been reports of improvement that occurred either spontaneously with renal recovery or after renal transplantation. We present an interesting case of a 33-year-old woman diagnosed with nephrogenic fibrosing dermopathy, along with a review of the literature.