Scleroderma-like acute renal crisis in a patient with scleromyxedema

Gwinner, Wilfried; Erdbruegger, Uta; Mengel, Michael; Hafer, Carsten; Kittner, Jens M.; Witte, Torsten; Voelker, B.; Haller, Hermann

doi:10.1093/ndt/gfm103

Cited by 6 publications

(2 citation statements)

References 14 publications

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“…Gwinner et al reported the case of another patient with scleromyxoedema who was admitted for severe hypertension and acute renal failure requiring haemodialysis. A renal biopsy showed thrombotic microangiopathy and mucin deposition in the vessel walls 6. Peeters et al reported the case of a patient with scleromyxoedema who developed renal failure with a renal biopsy revealing mucin deposition in the arteriolar walls and ischaemic glomerular changes 7.…”

Section: Discussionmentioning

confidence: 99%

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis

2022

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Scleromyxoedema is a rare mucinosis that primarily affects the skin. It is associated with monoclonal gammopathy and has many extracutaneous manifestations, however, renal involvement is rare. We report the case of a woman with monoclonal gammopathy and scleromyxoedema presenting with progressive exertional dyspnoea and acute renal failure. Workup of her renal failure revealed monoclonal gammopathy associated C3 glomerulonephritis. She was treated with intravenous steroids and discharged with plans to pursue annual monoclonal gammopathy laboratory monitoring. Given the rarity of renal scleromyxoedema, careful investigation of extracutaneous manifestations and comorbidities is critical to discern the primary pathological process in patients with scleromyxoedema who develop renal insufficiency.

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Section: Discussionmentioning

confidence: 99%

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis

2022

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“…The distribution of skin involvement in this patient is not typical of scleredema. [7][8][9] Many of the features described in this case could be explained by scleromyxedema; however, the absence of skin papules, one of the four diagnostic criteria, makes this diagnosis unlikely. Several cases of scleromyxedema have been associated with multiple myeloma.…”

Section: Myeloma-related Skin Disordersmentioning

confidence: 90%

Case 8-2015

Wigley¹,

Friday

Shepard

et al. 2015

N Engl J Med

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Pr esentation of C a seDr. Naina Rastalsky (Medicine): A 68-year-old man with multiple myeloma was seen in the rheumatology clinic of this hospital because of increasing skin tightness, joint pain, and swelling of the hands and feet.The patient had been well until 2 years before this presentation, when anemia was noted on routine examination at another hospital. During the next 7 months, endoscopic and colonoscopic screening examinations were negative. Pathological examination of a bone marrow-biopsy specimen and aspirate revealed 30% plasma cells; flow-cytometric studies revealed an IgG lambda M component. A diagnosis of multiple myeloma was made. Skeletal radiographs reportedly revealed multiple lytic lesions. Lenalidomide, bortezomib, and dexamethasone were administered, followed by cyclophosphamide. Nine months before this presentation, the patient was admitted to this hospital; melphalan hydrochloride was administered, and autologous stem-cell transplantation was performed. Results of follow-up studies were consistent with complete remission.Three months before this presentation, the patient was seen by an orthopedist at this hospital for evaluation of low-back pain of 1 year's duration. Magnetic resonance imaging (MRI) of the lumbar spine, performed after the administration of intravenous gadolinium, revealed multilevel degenerative changes, multiple enhancing lesions in the lumbar spine and left iliac bone, and compression fractures of the first and second lumbar vertebrae, findings consistent with the history of multiple myeloma.Two months before this presentation, swelling and pain in the hands occurred, followed by pedal edema, tightening of the skin of the hands and feet, and diffuse hyperpigmentation on the trunk, arms, and legs. Maintenance therapy with lenalidomide was begun, but it was stopped during the first cycle because of worsening symptoms.Diffuse joint pain occurred and hyperpigmentation increased. One month before this presentation, on evaluation in the outpatient cancer center of this hospital,

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Diagnostik und Therapie des Hochdrucks bei Systemerkrankungen

2017

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Erratum Dieser Beitrag beinhaltet keine vom Autor durchge-führten Studien an Menschen oder Tieren. Open Access. Dieser Artikel wird unter der Creative Commons Namensnennung 4.0 International Lizenz (http://creativecommons.org/licenses/by/4.0/deed. de) veröffentlicht, welche die Nutzung, Vervielfäl-tigung, Bearbeitung, Verbreitung und Wiedergabe in jeglichem Medium und Format erlaubt, sofern Sie den/die ursprünglichen Autor(en) und die Quelle ordnungsgemäß nennen, einen Link zur Creative Commons Lizenz beifügen und angeben, ob Änderungen vorgenommen wurden.

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Scleroderma-like acute renal crisis in a patient with scleromyxedema

Cited by 6 publications

References 14 publications

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis

Renal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis

Case 8-2015

Diagnostik und Therapie des Hochdrucks bei Systemerkrankungen

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