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CEN Case Reports

, Volume 6, Issue 2, pp 206–209 | Cite as

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

  • Shinsuke IsobeEmail author
  • Naro Ohashi
  • Naoko Katahashi
  • Sayaka Ishigaki
  • Naoko Tsuji
  • Takayuki Tsuji
  • Akihiko Kato
  • Yoshihide Fujigaki
  • Akira Shimizu
  • Hideo Yasuda
Case Report

Abstract

Cutaneous and systemic plasmacytosis (CSP) is a rare lymphoproliferative disorder that mainly affects middle-aged Asian individuals. Although Castleman disease is often complicated with various renal involvements, glomerulonephritis associated with CSP, which is considered as a variant of Castleman disease, is rare. This report presents the case of a 41-year-old Japanese man with nephrotic syndrome associated with CSP. Renal biopsy findings showed focal segmental glomerulosclerosis (FSGS) and diffusely mild segmental mesangial proliferation. Plasma cell infiltration in the interstitium was not observed. Electron microscopic findings showed diffuse foot process effacement, localized involvement of subendothelial space widening with amorphous materials, and endothelial cell swelling. Lymph node biopsy findings denied Castleman disease. His skin regions and proteinuria were successfully treated with prednisolone and cyclosporine. The causal relationship between CSP and FSGS is unknown. However, increased serum levels of IL-6 and VEGF and decreased VEGF expression in the podocyte may contribute to renal lesions in patients with CSP. To our best knowledge, this is the first case of a patient with FSGS associated with CSP.

Keywords

Focal segmental glomerulosclerosis Nephrotic syndrome Cutaneous and systemic plasmacytosis Castleman disease 

Notes

Compliance with ethical standards

Sources of funding

None.

Conflict of interest

All the authors have declared no competing interest.

Human rights

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

Written informed consent was obtained from the patient.

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Copyright information

© Japanese Society of Nephrology 2017

Authors and Affiliations

  1. 1.Internal Medicine 1Hamamatsu University School of MedicineHamamatsuJapan
  2. 2.Blood Purification UnitHamamatsu University School of MedicineHamamatsuJapan
  3. 3.Department of Internal MedicineTeikyo University School of MedicineTokyoJapan
  4. 4.Department of PathologyNihon University School of MedicineTokyoJapan

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