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Nephrogenic fibrosing dermopathy


Nephrogenic fibrosing dermopathy

Nephrogenic fibrosing dermopathy

الإعتلال الجلدي المليف كلوي المنشأ: تشخيص تفريقي يجب أن يدخل في بالنا عند آفة جلدية لدى مريض قصور كلية مزمن حالي أوسابق..

Intro:

Nephrogenic systemic fibrosis is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness

Nephrogenic systemic fibrosis manifests with induration, thickening, and hardening of the skin with brawny hyperpigmentation. Distinct papules and subcutaneous nodules can also be seen. Cellulitis is commonly suspected. The skin can have a peau d'orange appearance, and plaques may have an amoeboid advancing edge. The skin is often shiny and hard to the touch. A woody consistency is typical.


Physical appearance

The extremities are the most common areas of involvement, followed by the trunk. The face is almost never involved. Yellow palmar papules resembling cutaneous calcinosis have been reported. In addition, yellow scleral plaques have been reported in patients with nephrogenic systemic fibrosis. Solomon et al[36] noted nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma.


Causes:

The cause of nephrogenic systemic fibrosis is the connexation of renal insufficiency and gadolinium exposure from imaging studies. The exact degree of renal insufficiency that sets up the development of nephrogenic systemic fibrosis is not known. Risk factors include advanced chronic kidney disease (stages 4 and 5) and acute or chronic inflammatory insults. The US Food and Drug Administration (FDA) has updated its public health advisory to include patients with moderate renal insufficiency (chronic kidney disease stage 3).[37]


http://emedicine.medscape.com/article/1097889-overview


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