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Columbia International Publishing
American Journal of Contemporary Dermatology
(2014) Vol. 1 No. 1 pp. 1-5
Case Report
Nephrogenic Fibrosing Dermopathy; A Case Report
Reza M Robati1*, Somayeh Hejazi1, Zahra Asadi-kani1
Received 13 October 2013; Published online 8 March 2014
© The author(s) 2014. Published with open access at www.uscip.us
Abstract
Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis (NSF) was first recognized in 1997, is a
rare, diffuse, multisystem, fibrosing disease with an incidence of approximately 3–5% which affected men
and women equally and its etiology remains to be defined. We describe a 45-year-old Iranian woman on renal
transplantation who presented with multiple hypo and hyperpigmented, edematous, indurated, thickened
plaques of her upper and lower extremities started 3 months after kidney transplantation with diagnosis of
NFS.
Keywords: Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis; Renal failure; MRI
1. Introduction
Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis (NSF) is a rare, diffuse,
multisystem, fibrosing disease develops in the setting of advanced renal failure and characterized
with a wide ranges of manifestations, from only mild skin manifestation to severely debilitating
fibrosis that involving skin and other organs including the subcutaneous tissues, muscles, lungs,
pleura, pericardium, and bones.(Shabana et al., 2008;Robinson et al., 2011; Kribben et al.,2009).
In our patient, skin symptoms were relatively minor and were tolerated by the patient for as long
periods as 7 years without definitive evaluation.
2. Case report
A 45-year-old woman presented to our outpatient clinic with a complaint of multiple plaques on
her extremities which had been present since 7 years ago. Skin changes started on her left leg 3
months after renal transplantation, then spread to both legs, dorsal of foots, arms and dorsal aspect
of the hands with a slowly expanding course. The plaques were non-tender and non-pruritic.
______________________________________________________________________________________________________________________________
*Corresponding e-mail: [email protected]
1 Skin Research Center, Shahid Beheshti University of Medical Sciences,
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Shohadae-Tajrish Hospital, Tehran, Iran
Reza M Robati, Somayeh Hejazi, Zahra Asadi-kani /
American Journal of Contemporary Dermatology (2014) Vol. 1 No. 1 pp. 1-5
Physical examination revealed multiple symmetrical large hypo and hyperpigmented edematous
and mildly indurated plaques on her forearms, dorsal aspect of the hands, legs, ankles and dorsal of
foots with a pale and peau d’orange appearance [Figures1 and 2].
Fig 1. Multiple symmetrical large edematous and mildly indurated plaques on the dorsal
aspect of the hands, legs, ankles and dorsal of foots.
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Reza M Robati, Somayeh Hejazi, Zahra Asadi-kani /
American Journal of Contemporary Dermatology (2014) Vol. 1 No. 1 pp. 1-5
Fig 2. Large indurated plaques on the forearm with a pale and peau d’orange appearance
(closer view).
The joint examination showed normal range of motion, without sign of arthritis or muscle
contraction. The mucous membranes and the other areas of the skin were intact. She had no
evidence of systemic involvement or coexisting diseases, except positive history of hypertension
and diabetes mellitus. A history of administration of gadolinium-based contrast for imaging was
unremarkable for our patient.
Her family history was unknown . During these years, she has had neither evaluation nor
treatments for these lesions. Two skin biopsies were performed from her leg and her arm. [Figure
3] The differential diagnosis of myxedema, mucinosis and generalized granuloma annularis was
considered. Histopathological examinations revealed dense dermal fibrosis and sclerosis involved
dermis, septa of subcutis and fascia, increased dermal spindled cells with scattered multinucleated
giant cells compatible with clinical diagnosis of nephrogenic fibrosing dermopathy.
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Reza M Robati, Somayeh Hejazi, Zahra Asadi-kani /
American Journal of Contemporary Dermatology (2014) Vol. 1 No. 1 pp. 1-5
Fig 3. Dense dermal fibrosis and sclerosis (H&E*10)
3. Discussion
Nephrogenic fibrosing dermopathy characterized clinically by thickening and hardening of the skin
of the extremities and trunk with large brownish hyperpigmentated edematous, fibrotic or woody
plaques with or without pruritus and pain which may be mistaken for other fibrosing skin
disorders e.g. scleroderma, scleromyxoedema, systemic sclerosis/morphoea, pretibial myxedema,
eosinophilic fasciitis and eosinophilia myalgia syndrome. ( Kribben et al.,2009; Foss et al., 2009;
Mendichovszky et al., 2008)
At risk patients included who had a renal failure, a history of previous exposure to gadoliniumbased contrast material for MRI, especially in persons with higher dose of contrast exposure,
elevated plasma level of iron, calcium and phosphate, presence of chronic metabolic acidosis,
immunosuppression and vasculopathy, hypercoagulability, and tissue injury due to surgical
procedures.( Shabana et al.,2008; Perez-Rodriguez et al.,2009) Unfortunately, a history of
administration of gadolinium-based contrast before symptom onset was unknown for our patient,
but in whom nephrogenic systemic fibrosis developed 3 months after renal transplantation.
The diagnosis of NSF may be confirmed through the histopathological examination that may show
proliferation of fibroblast-like dermal cells, collagen remodeling, thickening and separation of
collagen bundles by clefts and variable amounts of mucin deposition. ( Kribben et al.,2009; Foss et
al., 2009; Mendichovszky et al., 2008' Song et al.,2009)
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Reza M Robati, Somayeh Hejazi, Zahra Asadi-kani /
American Journal of Contemporary Dermatology (2014) Vol. 1 No. 1 pp. 1-5
Although unusual, spontaneously resolution of skin lesions was reported especially in persons with
acute and transient renal failure. (Shabana et al., 2008)
According to the current knowledge, restoration of renal function by renal transplantation or
recovery from acute renal failure is the main therapeutic goal, but not effective in all patient.
(Kribben, et al., 2009) Other various therapeutic options that have been tried in the treatment of
NFS with different success rate included extracorporeal photopheresis, systemic corticosteroids,
plasmapheresis, ultraviolet phototherapy, intravenous immunoglobulin, thalidomide,
pentoxyphyllin, cyclophosphamide and the chelating agent.( Kribben et al., 2009; Mendichovszky et
al., 2008)
The findings in our case were similar to the findings of earlier experience with nephrogenic
systemic fibrosis that correlated with compromised renal function, but the strongly correlation
between gadolinium-based contrast administration and development of condition in this case was
difficult to establish.
Because of the risks of gadolinium-based contrast material in renal failure patients, MRI contrast
agents should be administrated with caution in these patients. It should be remembered that
diagnosis of NFS must be considered in cases of enlarging plaques in the setting of renal disorders.
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