We present a case of NSF occurred after gadolinium exposure in which the initial presentation mimics an erythema nodosum (EN)-like picture. An initial skin biopsy showed EN. Subsequently the patient developed progressive skin and joints contracture. [ncbi.nlm.nih.gov]

• Pain

  The patients reported significant improvements in subjective pain levels after TPE. Patient 1 reported decreased skin and contracture pain after the 3rd treatment, with similar results for a second series 6 months later. [ncbi.nlm.nih.gov]

  Go to the pain clinic. Healthcare providers at a pain clinic can help you learn new ways to control your pain. When should I contact my healthcare provider? Your arms or legs are swollen, or you have trouble moving them. [drugs.com]

  It is painful and often causes restricted mobility of the joints. In many cases, individuals diagnosed with nephrogenic systemic fibrosis become dependent on a wheelchair within a few weeks of the disease onset. [bernllp.com]

• Weakness

  BACKGROUND: A 61-year-old female with end-stage renal disease who was undergoing hemodialysis presented with an 8-week history of upper and lower extremity weakness associated with skin tightness and contractures. [ncbi.nlm.nih.gov]

  In severe cases, individuals diagnosed with NSF/NFD are unable to walk, unable to extend or move the joints of their arms, hands, legs or feet and complain of muscle weakness. [youhavealawyer.com]

  NSF presents with pain, pruritis, joint stiffness, tightness, swelling of the hands and feet, paraesthesias and burning. 2 Dry skin, weakness and warmth can also occur. [medsafe.govt.nz]

• Anemia

  Changes in bone marrow are likely secondary to anemia. Because of the uncommon nature of the meningeal abnormalities, the patient had a right frontal craniotomy and dural biopsy in May 2006. [ajnr.org]

  […] may lead to or promote the disease when severe kidney disease and exposure to gadolinium-containing contrast are present include: Use of high-dose erythropoietin ( EPO ), a hormone that promotes the production of red blood cells, often used to treat anemia [mayoclinic.org]

  The fact that these patients had significantly worse renal function as compared with most of the control subjects puts them at higher risk for requiring recombinant epoetin therapy for management of anemia. [emedicine.com]

• Leg Swelling

  The remaining six patients were more mildly affected (leg swellings and skin induration with/without local pain). [dx.doi.org]

  A 46-year-old woman with chronic renal failure, leg swelling, and skin changes. N Engl J Med 2009;361:2166-76. [Pubmed] [40] Gibson SE, Farver CF, Prayson RA. [revistanefrologia.com]

• Fatigue

  Physical examination revealed a frail, fatigued white man who was not in acute distress. [archderm.ama-assn.org]

  […] having recognized antifibrotic properties, is a drug of particular interest that I have found most effective in my own practice, but it is a difficult drug to use on those with many comorbidities, as it leads to side effects, including fluid-shifts and fatigue [dermatologyadvisor.com]

• Flexion Contracture

  NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. [ncbi.nlm.nih.gov]

• Myalgia

  In February 2003 the patient was admitted to another hospital because of a 4-week syndrome with fever, cough, dysnea and myalgias. [reumatologiaclinica.org]

  The differential diagnoses for NSF include diffuse cutaneous or limited cutaneous systemic sclerosis, scleromyxedema, lipodermatosclerosis, scleroedema diabeticorum, graft versus host disease, eosinophilic fasciitis; eosinophilia-myalgia syndrome; porphyria [en.wikipedia.org]

  Both the toxic oil syndrome and eosinophilia-myalgia syndrome demonstrate that toxins can cause cutaneous fibrosing disorders. 6 The CDC has begun collecting cases of NFD in an attempt to determine the underlying etiology. [archderm.ama-assn.org]

  Such agents include toxic/denatured rapeseed oil, tainted l -tryptophan supplements (the eosinophilia-myalgia syndrome), polyvinyl chloride, bleomycin, and carbidopa ( 10 ). [doi.org]

• Joint Stiffness

  NSF presents with pain, pruritis, joint stiffness, tightness, swelling of the hands and feet, paraesthesias and burning. 2 Dry skin, weakness and warmth can also occur. [medsafe.govt.nz]

  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 [emedicine.com]

  Within weeks, the second patient was unable to walk due to extreme muscle and joint stiffness. In 2003, this patient died from a pulmonary embolism. [ckj.oxfordjournals.org]

• Skin Thickening

  Skin thickening and tethering were assessed using the modified Rodnan skin thickness score (MRSS), and knee joint flexion contractures were measured with a goniometer. [ncbi.nlm.nih.gov]

  It is associated with scarring of the skin and connective tissue throughout the body, known as fibrosis. The skin thickens and becomes hard, rigid and coarse, which severely restricts movement of the joints. [bernllp.com]

• Hyperpigmentation

  NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. [ncbi.nlm.nih.gov]

  NSF presents with cutaneous hyperpigmentation and induration and joint contractures, but fibrosis may also develop in other organs. [ard.bmj.com]

• Skin Disease

  METHODS: Sixty English-speaking subjects were enrolled: 10 subjects diagnosed as having NSF, 10 subjects with other fibrosing skin diseases, 20 subjects with nonfibrosing skin diseases, and 20 subjects without a skin disease. [ncbi.nlm.nih.gov]

  Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin. [mayoclinic.org]

  A 68-year-old man with end stage renal disease and thickening of the skin. N Engl J Med 2004 ; 351 : 2219 –2227 20 Vicktor C., Schultz-Ehrenburg U. [doi.org]

  Urologic Diseases Skin Diseases [clinicaltrials.gov]

• Pruritus

  Eventually, flexure contractures develop, with restriction to movement, pain and pruritus. [radiopaedia.org]

  Many patients also noted pruritus and burning sensation in the affected skin. The clinical features of well established cutaneous lesions resembled the appearance of the cutaneous involvement of both systemic sclerosis and diffuse fasciitis. [ncbi.nlm.nih.gov]

  Additionally, many patients report maddening pruritus and/or causalgia. Finally, some patients experience flexion contractures if the disorder occurs over a joint. [emedicine.com]

  Southern California medical center were confronted with a mysterious clinical entity. 1,2 Several patients in their hemodialysis center began experiencing cutaneous induration and erythema of the limbs unassociated with fever, but frequently accompanied by pruritus [appliedradiology.com]

• Skin Plaque

  It is typically characterised by hard, erythematous and indurated skin plaques with surrounding subcutaneous oedema. Distinct papules and subcutaneous nodules can also be seen. Fibrocytes in NSF are immunohistochemically positive for CD34. [ncbi.nlm.nih.gov]

  The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. [reumatologiaclinica.org]

• Suggestibility

  Although this condition was initially felt to be limited to the skin, recent studies have described widespread involvement, and the term nephrogenic systemic fibrosis (NSF) has been suggested. [ncbi.nlm.nih.gov]

  In 2006, Grobner 9 suggested gadolinium as the cause of NSF. [ajnr.org]

• Renal Insufficiency

  METHODS: A study among GBCA-exposed patients with renal insufficiency (n=565) was conducted to identify cases of NSF. [ncbi.nlm.nih.gov]

  OMNISCAN, OPTIMARK and MAGNEVIST are contraindicated in: Patients with chronic severe renal insufficiency where glomerular filtration rate is 2 ; Patients with acute kidney injury (AKI) (including those with acute renal insufficiency of any severity due [healthycanadians.gc.ca]

  The strong association with renal insufficiency most likely relates to the prolonged biological half-life due to prolonged excretion of gadolinium. [mriquestions.com]

• Kidney Failure

  Nephrogenic systemic fibrosis (NSF) is a fibrosing skin disorder that develops in patients with kidney failure and has been linked to exposure to gadolinium-containing contrast agents. [ncbi.nlm.nih.gov]

  Based on reported cases currently available, it appears that about 5% of those with kidney failure or kidney problems experience the systemic disorder after receiving a gadolinium agent. [bernllp.com]

• Renal Function Impairment

  Repeated or higher than recommended doses of a GBCA and the degree of renal function impairment at the time of exposure are risk factors for NSF Footnote 4. [healthycanadians.gc.ca]

  At that time, the use of GBCA represented the possibility of avoiding the use of iodinated contrast media in patients who already had significant renal function impairment. [doi.org]

We propose that skin biopsies performed as part of a diagnostic workup for suspected NSF should preferably include the deep dermis and subcutis, in order to assess possible vascular calcification. [ncbi.nlm.nih.gov]

Cowper, Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations, Journal of the American Academy of Dermatology, 10.1016/j.jaad.2010.08.041, 65, 6, (1095-1106.e7), (2011). [doi.org]

Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations. J Am Acad Dermatol 2011;65(6):1095-106. [29] Kaewlai R, Abujudeh H. Nephrogenic systemic fibrosis. AJR Am J Roentgenol 2012;199(1):W17-23. [revistanefrologia.com]

• Pericardial Effusion

  An echocardiogram showed a light pericardial effusion with hypertrophy of the left ventricle and preserved function. An electromyogram showed data of myopathy. [reumatologiaclinica.org]

Because skin changes recurred after discontinuation of imatinib mesylate, the duration for which treatment may be required is undetermined. [ncbi.nlm.nih.gov]

Overall, the prognosis is poor and there is no established therapy that shows a consistent benefit. The purpose of this review is to discuss the clinical spectrum of the disease. [ncbi.nlm.nih.gov]

(Outcomes/Resolutions) The prognosis of Nephrogenic Systemic Fibrosis is generally poor despite suitable treatment The prognosis also depends on the severity of the underlying condition and the involvement of various internal organs Additional and Relevant [dovemed.com]

While its histologic characteristics have been well described, the etiology and pathogenesis have not been fully characterized. [ncbi.nlm.nih.gov]

In addition, epidemiologic data have yielded several distinct clinical patterns of disease onset. [ncbi.nlm.nih.gov]

This article reviews the history, clinical manifestations, epidemiology, histopathology and pathophysiology of this disabling disease. [ncbi.nlm.nih.gov]

Other than limiting exposure to gadolinium based contrast agents, no effective preventive methods have been reported. [ncbi.nlm.nih.gov]

(e) Consider performing IHD after the exposure (and the next 2 days) in patients who are already maintained on IHD, recognizing that there are no data that support prevention of NSF with this modality. [qxmd.com]