Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: clinical aspects

Abstract

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a novel fibrosing disorder characterized by prominent cutaneous and systemic fibrosis in patients with renal failure. Although initially descriptions of this disorder was described to be purely cutaneous, numerous subsequent reports have shown remarkable systemic involvement with fibrosis of numerous internal organs, including striated muscles, myocardium, microvasculature, lungs, and others, accompanied by elevation of systemic inflammation markers, such as erythrocyte sedimentation rate and C-reactive protein. The cutaneous manifestations are characterized by erythematous to browny skin induration with a peau d'orange appearance, plaques, nodules, and papules. Histopathologically, there is dermal and subdermal thickening with abundant collagen fibers and mucin deposits and a striking increase in the number of spindle-shaped fibroblastic cells. The disorder is clinically distinct from scleromyxedema, systemic sclerosis, and other cutaneous fibrosing syndromes. The cause is unknown, and currently there is no uniformly effective treatment.