Nephrogenic systemic fibrosis: Difference between revisions
(Created page with "==Background== *Gadolinium exposure (MRI with contrast) to pts with renal insufficiency (HD, PD, transplant) *Poorly understood pathogenesis *Chronic, progressive condition wi...") |
No edit summary |
||
| Line 43: | Line 43: | ||
==Sources== | ==Sources== | ||
*Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview. | |||
<references/> | <references/> | ||
[[Category:FEN]] | [[Category:FEN]] | ||
Revision as of 22:19, 2 May 2016
Background
- Gadolinium exposure (MRI with contrast) to pts 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
- Hx 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
- 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
Treatment
- Treatments anechdotal 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
Sources
- Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.