Nephrogenic fibrosing dermopathy with systemic involvement.

BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. OBSERVATIONS: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. CONCLUSION: A subset of patients with NFD may have significant systemic involvement.

Practical and experimental consideration of sun protection in dermatology.

Much is known regarding the deleterious effects of ultraviolet radiation (UV) on the skin. As more epidemiologic and basic research continues to characterize the impact of sun exposure and other sources of UV radiation upon the development of cutaneous neoplasm and a variety of photosensitive dermatoses, it is crucial for the dermatologist to promote sun protection among his/her patients as well as the primary care physician who has a greater reach of the community than the skin specialist. Practical steps to achieve optimal sun protection include avoidance of UV radiation, avoidance of photosensitizing drugs, use of photo-protective clothing, and diligent application of broad-spectrum sunscreens. In recent years, novel agents and experimental modalities with the potential to offer enhanced protective effects against deleterious sequelae of sun exposure have been elucidated, e.g. antioxidants, alpha-MSH, polyphenol in green teas, genistein, NF-kB decoy oligodeoxynucleotides, pTpT vaccination, and IL-12. As these new photo-protective tools are being developed by scientists around the world, greater concerted effort is needed to engage public health officials and the media to promote sun protection awareness throughout the general public.