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.
This is a preview of subscription content, log in to check access.
Compliance with ethical standards
Sources of funding
Conflict of interest
All the authors have declared no competing interest.
This article does not contain any studies with human participants performed by any of the authors.
Written informed consent was obtained from the patient.
Yashiro A. A kind of plasmacytosis: primary cutaneous plasmacyoma? Jpn J Dermatol. 1987;86:910.Google Scholar
Watanabe S, Ohara K, Kukita A, et al. Systemic plasmacytosis. A syndrome of peculiar multiple skin eruption, generated lymphadenopathy, and polyclonal hypergammaglobulinemia. Arch Dermatol. 1986;122:1314–20.CrossRefGoogle Scholar
Haque M, Hou JS, Hisamichi K, et al. Cutaneous and systemic plasmacytosis vs. cutaneous plasmacytic Castleman disease: review and speculations about pathogenesis. Clin Lymphoma Myeloma Leuk. 2011;11:453–61.CrossRefGoogle Scholar
Kayasut K, Le Tourneau A, Rio B, et al. Are multicentric Castleman’s disease with cutaneous plasmacytosis and systemic plasmacytosis the same entity? Histopathology. 2006;49:557–8.CrossRefGoogle Scholar
El Karoui K, Vuiblet V, Dion D, et al. Renal involvement in Castleman disease. Nephrol Dial Transplant. 2011;26:599–609.CrossRefGoogle Scholar
Xu D, Lv J, Dong Y, et al. Renal involvement in a large cohort of Chinese patients with Castleman disease. Nephrol Dial Transplant. 2012;27(Supple 3):iii119–25.CrossRefGoogle Scholar
Toda Y, Komine M, Suzuki S, et al. Plasmacytosis: systemic or cutaneous, are they distinct? Acta Dermato Venereol. 2000;80:233–5.Google Scholar
Kodama A, Tani M, Hori K, et al. Systemic and cutaneous plasmacytosis with multiple skin lesion and polyclonal hypergammaglobulinaemia: significant serum interleukin-6 levels. Br J Dermatol. 1992;127:49–53.CrossRefGoogle Scholar
Fogo AB. Causes and pathogenesis of focal segmental glomerulosclerosis. Nat Rev Nephrol. 2015;11:76–87.CrossRefGoogle Scholar
Dingli D, Larson DR, Plevak MF, et al. Focal and segmental glomerulosclerosis and plasma cell proliferative disorder. Am J Kidney Dis. 2005;46:278–82.CrossRefGoogle Scholar
Bower M, Stebbing J. Exploring interleukin 6 in multicentric Castleman’s disease. Lancet Oncol. 2014;15:910–2.CrossRefGoogle Scholar
Higashi Y, Kanekura T, Sakamoto R, et al. Multicentriic Castleman disease with cutaneous manifestations: report of 2 cases and comparison with systemic plasmacytosis. Dermatology. 2007;214:170–3.CrossRefGoogle Scholar
Yuan XG, Hu W, Chen FF, et al. Renal complications of Castleman’s disease: report of two cases and analysis of 75 cases. Clin Exp Nephrol. 2011;15:921–6.CrossRefGoogle Scholar
Brandt SJ, Bodine DM, Dunbar CE, et al. Dysregulation interleukin 6 expression produces a syndrome resembling Castleman’s disease in mice. J Clin Invest. 1990;86:592–9.CrossRefGoogle Scholar
Chen WP, Chen A, Lin CY. In vitro effects of interleukins on human mesangial cells: implications for glomerulonephritis. J Pathol. 1995;175:327–37.CrossRefGoogle Scholar
Henao DE, Saleem MA, Cadavid AP. Glomerular disturbances in preeclampsia: disruption between glomerular endothelium and podocyte symbiosis. Hypertens Pregnancy. 2010;29:10–20.CrossRefGoogle Scholar
Wang L, Bu D, Yang Y, et al. Castleman’s tumors and production of autoantibody in paraneoplastic pemphigus. Lancet. 2004;363:525–31.CrossRefGoogle Scholar