Nephrogenic Systemic Fibrosis

1. Nephrogenic Systemic Fibrosis Chow YW Department of Nephrology, Kuala Lumpur Hospital

5. Nephrogenic Systemic Fibrosis Epidemiology

8. Nephrogenic Systemic Fibrosis Etiology

14. RISK ====  5% (advance CKD) Danish Med Agency. 2006

18. Pathology

21. Sufficiently deep biopsies show increased numbers of fibroblast-like cells in the dermis, extending into the fascia along subcutaneous septa.

22. The dermis demonstrates haphazardly arranged collagen bundles and a strikingly increased number of spindled and plump fibroblast-like cells

23. The spindled and plump fibroblast-like cells extend into the fascia along subcutaneous septa.

24. Mucin is frequently present and demonstrable by the colloidal iron or alcian blue (pH 2.5) staining methods

25. Small multinucleated histiocytes may also be a feature.

27. Clinical Manifestation

31. Areas of thick, hardened skin, commonly associated with brawny hyperpigmentation, and preferentially localized to the extremities

32. Extensive thickening of the skin, often associated with brawny hyperpigmentation, and in some cases, distinct papules and subcutaneous nodules.

35. Diagnosis

44. Treatment