The International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR)
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By Shawn E. Cowper, MD
Associate Professor of Dermatology and Pathology
Areas of thick, hardened skin, commonly associated with brawny hyperpigmentation, and preferentially localized to the extremities.
Extensive thickening of the skin, often associated with brawny hyperpigmentation, and in some cases, distinct papules and subcutaneous nodules.
Sufficiently deep biopsies show increased numbers of fibroblast-like cells in the dermis, extending into the fascia along subcutaneous septa.
The dermis demonstrates haphazardly arranged collagen bundles and a strikingly increased number of spindled and plump fibroblast-like cells.
The spindled and plump fibroblast-like cells extend into the fascia along subcutaneous septa.
Mucin is frequently present and demonstrable by the colloidal iron or alcian blue (pH 2.5) staining methods
Small multinucleated histiocytes may also be a feature.
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