Nephrogenic systemic fibrosis: late skin manifestations.
BACKGROUND
Nephrogenic systemic fibrosis (NSF) is a serious disease that occurs in patients with severe renal disease and is believed to be caused by gadolinium-containing contrast agents. A detailed description of the late skin manifestations of NSF is important to help dermatologists and nephrologists recognize the disease.


METHODS
We studied 17 patients with NSF late in the disease. All patients showed epidermal atrophy and hairlessness of the affected regions, primarily the lower legs. Affected areas were symmetrically distributed and hyperpigmented in most cases. Eleven patients showed confluent dermal plaques with thickening and hardening. In contrast, 3 patients presented with wrinkled, redundant skin as seen in cutis laxa. Patients with NSF had significantly poorer scores than control patients on the Daily Life Quality Index (mean [SD], 11. 4 [7.4] vs 1.5 [2. 3]; P < .001).


CONCLUSIONS
This descriptive case series of patients with NSF gives a detailed clinical picture of the skin manifestations late in the disease. It demonstrates that the clinical picture in the late stage has a varied presentation and that NSF has a significant effect on the quality of life.
Bangsgaard
Nannie
N
Department of Dermatology, University Hospital of Copenhagen Gentofte, Niels Andersens Vej 65, DK-2900 Hellerup, Denmark. Nanban01@geh.regionh.dk
Marckmann
Peter
P
Rossen
Kristian
K
Skov
Lone
L
D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D015331:Cohort Studies; D003287:Contrast Media / Q000009:adverse effects; D005260:Female; D019786:Gadolinium DTPA / Q000009:adverse effects; D006801:Humans; D008297:Male; D008875:Middle Aged; D054989:Nephrogenic Fibrosing Dermopathy / Q000097:blood / Q000453:epidemiology* / Q000473:pathology; D010446:Peptide Fragments / Q000097:blood; D011347:Procollagen / Q000097:blood; D011788:Quality of Life; D051437:Renal Insufficiency / Q000097:blood / Q000150:complications / Q000473:pathology*; D012871:Skin Diseases / Q000097:blood / Q000453:epidemiology* / Q000473:pathology
D003287:Contrast Media; D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide; C064925:gadodiamide; D019786:Gadolinium DTPA