A 75yearold woman with endstage renal disease on hemodialysis presented with a 2month history of progressive skin thickening of the lower extremities. Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of skin, joints, eyes, and internal organs. Nephrogenic fibrosing dermopathy nfd is an acquired, idiopathic, chronic, progressive eruption of the skin sometimes accompanied by systemic fibrosis that occurs in the context of renal failure. Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin. Nsf is caused by exposure to gadolinium in gadoliniumbased mri contrast agents gbcas in patients with impaired kidney function. Nephrogenic fibrosing dermopathy nfd is a progressive disorder which has been shown to occur in those with chronic kidney problems or impaired renal function. It was first described in patients with kidney disease in 1997. Nephrogenic fibrosing dermopathy was first noticed clinically in 1997. Pediatric nephrogenic systemic fibrosis is rarely reported.
Most patients with nfd have undergone dialysis for renal failure 1,2. However, in 1997, a new disease emerged, originally called nephrogenic fibrosing dermopathy. Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens. Gadolinium a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis. Byung chul kang1 1chung ang university hospital, seoul, korea purpose. Nephrogenic fibrosing dermopathynephrogenic systemic fibrosissetting the. The age of patients with nfd ranges from 8 to 87 years at the time of onset, with a mean age of 46. Observational study on the incidence of nephrogenic systemic fibrosis in renal impaired patients following gadoterate meglumine administration. Nephrogenic fibrosing dermopathy associated with exposure. Gadoliniuma specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis.
Louis, missouri, 20022006 nephrogenic fibrosing dermopathy nfd causes thickening and hardening of the skin, often in the extremities, and occurs in patients with underlying renal disease. Nephrogenic fibrosing dermopathynephrogenic systemic. Minimizing risk of nephrogenic systemic fibrosis in. Gadolinium deposition in nephrogenic fibrosing dermopathy. Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, is associated with the formation of excessive scar tissue in the skin and connective tissue of other internal organs. Apr 24, 20 nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin. Nephrogenic fibrosing dermopathy associated with exposure to gadoliniumcontaining contrast agents st. Nephrogenic fibrosing dermopathy associated with exposure to. Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to. Gadoliniumassociated nephrogenic systemic fibrosis.
Nephrogenic fibrosing dermopathy is a recently described fibrosing disorder that emerges in different settings of renal insufficiency. Oct 01, 2009 nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadoliniumbased contrast agents. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy nfd, is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. Later this entity was renamed nephrogenic systemic fibrosis nsf when involvement of internal organs was discovered. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and. Laboratory values at admission are shown in table 1. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and histologically by an increase in spindle. Dialysisassociated systemic fibrosis nephrogenic fibrosing dermopathy. Nephrogenic systemic fibrosisnephrogenic fibrosing dermopathy. Nephrogenic fibrosing dermopathy lawsuits lawyers attorneys. Nephrogenic fibrosing dermopathynephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure.
The cause of nephrogenic systemic fibrosis is attributed to the connexation of renal insufficiency and. This results in thick, coarse and hard skin, which make it difficult to move the arms, hands, legs andor feet. Nephrogenic fibrosing dermopathy nfd is a rare condition in which there is a sudden onset of nodular or plaquelike skin lesions. It is very common for renal patients to have undergone a vascular procedure, such as av fistula formation before starting haemodialysis. Nephrogenic fibrosing dermopathy, also known as nephrogenic systemic fibrosis, is a recently described acquired, idiopathic disorder that manifests in patients with renal disease. Nephrogenic fibrosing dermopathy in children springerlink. Previously called nephrogenic fibrosing dermopathy case reports 61 year old woman with nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma arch pathol lab med 2007. Recently described systemic fibrosing disorder primarily affecting patients with chronic renal insufficiency exposed to gadolinium based contrast agents clin j am soc nephrol 2010. Gadoliniuma specific trigger for the development of. Eventually, flexure contractures develop, with restriction to movement, pain. It is a condition normally found in people with kidney disease.
Many endogenous as well as exogenous factors have been implicated in the. Nephrogenic fibrosing dermopathy nfd is an entity of unknown etiology that has only been reported in patients with impaired kidney function. Nephrogenic systemic fibrosis nsf nephrogenic fibrosing dermopathy nfd is a. Pdf gadoliniumassociated nephrogenic systemic fibrosis. Pdf nephrogenic systemic fibrosis nsf is a multisystem disease seen. Dec 06, 2019 nephrogenic fibrosing dermopathynephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure.
Although initially recognized as a cutaneous disorder and named nephrogenic fibrosing dermopathy, extensive systemic involvement has been identified in patients with nsf, including fibrosis of lymph nodes, thyroid, esophagus, heart, lungs, liver, diaphragm, skeletal muscle, genitourinary tract, and dura mater 7, 8. Nephrogenic systemic fibrosis symptoms and causes mayo clinic. West of scotland experience of nephrogenic systemic fibrosis in. Abraham, mdc nashville, tennessee, and syracuse, new york there is growing recognition of the association between the use of gadoliniumcontaining radiocontrast. Since then, more than 360 cases have been tracked by the yale international nsf registry 2 and over 500 cases have been reported to the. Nephrogenic systemic fibrosis nsf is a rare, potentially fatal condition caused by iatrogenic gadolinium administration in patients with acute kidney injury or stage 4 or 5 chronic kidney disease ckd, defined as an estimated glomerular filtration rate egfr of less than 30 mlmin1. Pdf gadolinium deposition in nephrogenic fibrosing. The condition can resemble scleroderma in appearance.
Symptoms of the condition may include progressive swelling and tightening of the skin, sometimes resulting in contractures, and pruritis itching. Nephrogenic systemic fibrosis is a disorder primarily affecting middleaged adults, although there is no racial or sex predilection for this disorder. They searched pubmed from january 2000 to february 2019 according to the key words nephrogenic systemic fibrosis, nsf and nephrogenic fibrosing dermopathy. Nephrogenic systemic fibrosis an overview sciencedirect. The exact pathogenesis of nephrogenic systemic fibrosis is unclear, but the disease has been linked with the use of gadoliniumbased contrast agents, predominantly in patients with acute renal failure or. Nephrogenic systemic fibrosis what you need to know. Nephrogenic systemic fibrosis nsf is a disorder seen only in patients with advanced kidney disease and is characterized by two primary features. Eventually, flexure contractures develop, with restriction to movement, pain and pruritus. Nephrogenic systemic fibrosis nsf is the thickening and hardening of your skin, mostly on your arms and legs. Nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin. Nephrogenic fibrosing dermopathy is a rare condition that results in tightened, swollen skin. Risk of nephrogenic systemic fibrosis in patients with stage.
Nephrogenic systemic fibrosis nsf, previously called nephrogenic fibrosing dermopathy, is an acquired, progressive, systemic fibrosing disorder of unknown etiology that develops in the setting of renal disease. A case of nephrogenic fibrosing dermopathynephrogenic. Cutaneous changes of nephrogenic systemic fibrosis. Nephrogenic systemic fibrosis nsf, previously known as nephrogenic fibrosing dermopathy, is an emerging systemic fibrosing disorder that develops in the setting of renal insufficiency. Nephrogenic systemic fibrosis nsf, also known as nephrogenic fibrosing dermopathy, is a complication of gadoliniumbased contrast agents used in mri. Grobner t 2006 erratum gadolinium a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis. Nephrogenic systemic fibrosis radiology reference article. Nephrogenic fibrosing dermopathy has also been seen in patients with clotting problems or who have had recent surgery particularly vascular surgery. Background nephrogenic fibrosing dermopathynephrogenic systemic fibrosis nfdnsf is a fibrosing cutaneous disorder recently recognized to have systemic manifestations. It is characterized by firm, erythematous, and indurated plaques of the skin associated with subcutaneous edema 1.
Nephrogenic systemic fibrosis is a recently recognized disease entity that is potentially debilitating. It is chiefly seen in patients of chronic kidney disease, those on hemodialysis and those who have had a history of exposure to gadoliniumbased contrast media. The purpose of this article is to discuss nephrogenic systemic fibrosis nsf in detail regarding its history, possible pathophysiology, clinical and pathologic presentations, diagnosis, and implications for the radiology community. The skin findings are similar to those seen in patients with scleroderma. Nephrogenic systemic fibrosis clinical presentation. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent. Nephrogenic systemic fibrosisnephrogenic fibrosing dermopathy in. It is caused by gadolinium exposure used in imaging in patients who have renal insufficiency. Nephrogenic fibrosing dermopathy is an acquired sclerodermalike. Gadolinium deposition and nephrogenic systemic fibrosis. Feb 03, 2020 what is nephrogenic systemic fibrosis. Nephrogenic fibrosing dermopathy in pediatric patients. The exact pathogenesis of nephrogenic systemic fibrosis is unclear, but the disease has been linked with the use of gadoliniumbased contrast agents, predominantly in patients with acute renal failure or endstage renal disease.
Nephrogenic systemic fibrosis nsf was originally called nephrogenic fibrosing dermopathy because of its predilection for extensive cutaneous involvement, but the newer term is more apt, because the condition involves extracutaneous tissues. Nephrogenic fibrosing dermopathy nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure. Risk of nephrogenic systemic fibrosis in patients with. It was first described by cowper in 2001 and the relationship to gbca exposure has been strongly suspected since 2006 37. Gadolinium a specific trigger for the development of. Nephrogenic systemic fibrosis nsf was first recognized in 1997 and. Furthermore, they extracted the risk factors, clinical data, outcomes and possible treatment in patients with biopsyverified nsf. Theearliestknowncaseofnsf developed in january 1997. May 22, 2018 kucher c, steere j, elenitsas r, siegel dl, xu x. Most cases have been reported in the us and europe, but recently cases have also been. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in.
Nephrogenic systemic fibrosis nsf is a rare disease involving severe thickening and hardening of the skin fibrosis overlying the extremities and trunk. The link between nephrogenic systemic fibrosis nsf and gadoliniumbased contrast agents gbcas was made in 20061,2. Skin thickening, tightness and contractures are hallmarks of the clinical findings in nephrogenic fibrosing dermopathy. Nephrogenic fibrosing dermopathynephrogenic systemic fibrosissetting the record straight. Diagnostic tests that are performed for nephrogenic fibrosing dermopathy may include. Nephrogenic fibrosing dermopathy predominantly affects the skin, but abnormal fibrosis of the internal organs may also occur, which is then often called nephrogenic systemic fibrosis. Your muscles, joints, organs, and the tissue that covers your brain may also be affected. Symptoms nephrogenic systemic fibrosis diagnosis nsf.
Clinically, these patients present with sclerodermalike plaques often involving the extremities. Epidemiological studies suggest that the incidence of nsf is unrelated to gender or ethnicity and it is not thought to have a genetic basis. Nephrogenic systemic fibrosis nsf, initially named nephrogenic fibrosing dermopathy, was first reported in 2001bycowperetal. The degree and cause of the underlying renal impairment seem not to be related to the severity of the nephrogenic fibrosing dermopathy. Marckmann p, skov l, rossen k, dupont a, damholt mb, heaf jg. Nephrogenic fibrosing dermopathy nfd is an acquired, idiopathic disorder that is observed in patients with renal disease. Nephrogenic systemic fibrosis nsf is a rare disease that occurs in patients with renal failure. It has characteristic diagnostic findings by skin biopsy and due to its systemic involvement in some reported cases, the term nephrogenic systemic fibrosis has been recently suggested as more appropriate for the nomenclature of this entity. The first trial over nephrogenic systemic fibrosis nsf from omniscan, a gadoliniumbased mri contrast agent manufactured by ge healthcare, was settled in a.
Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. Nephrogenic fibrosing dermopathy bahar f firoz md mph, christopher m hunzeker md, anthony c soldano md, andrew g franks jr md dermatology online journal 14 5. Nephrogenic systemic fibrosis genetic and rare diseases. The cutaneous changes consisted largely of indurated plaques and papules on the extremities and trunk. Nephrogenic fibrosing dermopathy with systemic involvement. Nephrogenic systemic fibrosis is a rare condition, and cases should be reported to the international center for nephrogenic fibrosing dermopathy research at yale.
Nephrogenic systemic fibrosis fulltext american journal of. Nephrogenic systemic fibrosisnephrogenic fibrosing. Nephrogenic systemic fibrosis is a recently recognized disease entity that is. It is associated with fibrosis, or scarring, which results in thick, coarse and hard skin around the joints, which can severely restrict movements.
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