HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, idiopathic, acquired, acutely onset, sclerosing disease of the skin, subcutaneous fatty tissue and musculature (more rarely is involvement of internal organs) which occurs almost exclusively in patients with advanced chronic renal insufficiency (GFR<30ml/min/1.73m2) after administration of gadolinium-containing contrast media (gadodiamide, gadopentetate dimeglumin).
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Occurrence/EpidemiologyThis section has been translated automatically.
Rare; currently about 200 cases have been reported in world literature, although a significantly higher incidence can be assumed. The incidence is clearly increasing. No gender preference (in contrast to systemic scleroderma).
EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Integument: Plaque-like and/or diffuse, board-hard (woody) thickening and induration of the skin, subcutis and underlying muscles with brownish, yellowish discoloration. Occasional papules and subcutaneous nodules. Skin lesions begin on the extremities, then spread to the trunk. Face usually excluded. Frequent flexion contractures in the course with need for a wheelchair. Burning pain, itching.
Extracutaneous manifestations: weakness, muscle pain. Extensive fibrosis and calcification of the diaphragm, the psoas muscle and the kidneys have been observed in isolated cases.
HistologyThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
|Scleromyxoedema||nephrogenic systemic fibrosis|
|Face mostly affected||Face free|
|Paraproteins in serum||no paraproteins in serum|
|Plasma cells in the infiltrates||No plasma cells|
|Mucin multiplied||no mucin|
|Fibroblasts reduced||Fibroblasts increased|
TherapyThis section has been translated automatically.
Radiation therapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
Note(s)This section has been translated automatically.
LiteratureThis section has been translated automatically.
- Baron PW et al (2003) Nephrogenic fibrosing dermopathy after liver transplantation successfully treated with plasmapheresis. Am J Dermatopathol 25: 204-209
- Cowper SE, Bucala R (2003) Nephrogenic fibrosing dermopathy: suspect identified, motive unclear. Am J Dermatopathol 25: 358
- Galan A et al (2006) Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol 18: 614-617
- Haemel AK et al (2011) Update on nephrogenic systemic fibrosis: are we making progress? Int J Dermatol 50:659-666
- High WA (2007) Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol 56: 21-26
- Kobus S et al (2010) Nephrogenic systemic fibrosis-10 cases from the Bochum clinic. JDDG 11: 935
- LeBoit PE (2003) What nephrogenic fibrosing dermopathy might be. Arch Dermatol 139: 928-930
- Mackay-Wiggan JM et al (2003) Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol 48: 55-60.
- Ortega LM et al.(2009) Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis? What do we know and what do we have to learn?. Nefrologia 29:109-117.
- Richmond H et al. (2007) Nephrogenic systemic fibrosis: relationship to gadolinium and response to photopheresis. Arch Dermatol 143: 1025-1030
- Ting WW et al (2003) Nephrogenic fibrosing dermopathy with systemic involvement. Arch Dermatol 139: 903-906
- Swartz RD et al (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 114: 563-572
Incoming links (9)Contrast medium intolerance; Fibrosis nephrogenic systemic; Gadolinium; Induration; Melphalan; Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis; Pseudoscleroderma; Scleromyxoedema;
Outgoing links (11)Acitretin; Circumscripts of scleroderma (overview); Eosinophilic myalgie syndrome; Fasciitis eosinophil; Gadolinium; Melphalan; Photopheresis extracorporeal; Plasmapheresis; Scleroderma systemic; Scleromyxoedema; ... Show all
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