Nephrogenic systemic fibrosis: Difference between revisions
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==Clinical Features== | ==Clinical Features== | ||
* | *History of HD, PD, renal transplant | ||
*Gadolinium exposure, with timeline of symptom onset variable | *Gadolinium exposure, with timeline of symptom onset variable | ||
*Tightened, thickened, shiny skin | *Tightened, thickened, shiny skin | ||
Revision as of 05:35, 15 July 2016
Background
- Gadolinium exposure (MRI with contrast) to patients with renal insufficiency (HD, PD, transplant)
- Poorly understood pathogenesis
- Chronic, progressive condition with rare cases of resolution in cases of return of renal function
Clinical Features
- History of HD, PD, renal transplant
- Gadolinium exposure, with timeline of symptom onset variable
- Tightened, thickened, shiny skin
- Peau d'orange appearance
- Hand stiffening
- Flexion contractures, severe mobility issues
- Extremities more commonly affected than trunk
- Face is almost never involved
- Associations:
Differential Diagnosis
- Scleroderma, morphea
- Calciphylaxis, which may coexist
- Cellulitis
- Necrotizing fasciitis
- Porphyria
- Eosinophilic fasciitis
Workup
- No particular lab or imaging diagnostics
- Deep skin biopsy, including dermis, subQ fat, fascia
Management
- Treatments anecdotal and of minimal benefit
- Extracorporeal photopheresis (ECP)
- UV phototherapy
- Immunotherapy
- No surgical role except renal transplant
Disposition
- Dermatologist for deep skin bx
- Nephrologist for renal disease management
- Does not inherently require inpatient care
See Also
References
- Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.