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March 20, 2007

Nephrogenic Systemic Fibrosis Mimicking Inflammatory Breast Carcinoma

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a newly recognized systemic fibrosing disorder primarily affecting patients with chronic renal failure. Patients with skin involvement often develop papules and plaques with peau d'orange surface changes. The lower extremities and trunk are most commonly affected. The most important histologic differential diagnosis is with scleromyxedema. This is possibly the first report of a case of nephrogenic systemic fibrosis involving the breasts of a 61-year-old woman with end-stage renal disease. Clinically, it mimics inflammatory breast carcinoma. Nephrogenic systemic fibrosis (NSF) is a recently described systemic fibrosing disorder primarily affecting patients with chronic renal insufficiency. Although the exact cause is unknown, bone marrow–derived circulating fibrocytes are suspected to be involved in the pathogenesis of this rare condition. Skin involvement in NSF frequently presents on the extremities as erythematous papules or plaques, often with peau d'orange surface changes and woody induration. Upper body involvement is less common, and breast involvement has not previously been reported.

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