Systemic plasmacytosis. A syndrome of peculiar multiple skin eruptions, generalized lymphadenopathy, and polyclonal hypergammaglobulinemia

Watanabe, Shaw

doi:10.1001/archderm.122.11.1314

Cited by 47 publications

(69 citation statements)

References 18 publications

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“…CSP affects mainly middle-aged Asian patients, particularly Japanese individuals. In most cases, the skin, bone marrow, and lymph nodes are involved in this disease [2]. Although the etiology of CSP has not been clearly elucidated, increased secretion of interleukin 6 (IL-6) from mature plasma cells in the skin to circulation plays a pivotal role in its pathogenesis [3].…”

Section: Introductionmentioning

confidence: 99%

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

et al. 2017

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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.

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Section: Introductionmentioning

confidence: 99%

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

et al. 2017

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“…Hauptsächlich betroffen sind die Isotypen IgG und IgA, deutlich seltener und weniger ausgeprägt das IgM [3,16,21]. Die Höhe der Hypergammaglobulinämie wurde von Uhara et al als Prognosefaktor angesehen.…”

Section: Extrakutane Manifestationunclassified

“…Zu den Organkomplikationen zählen die Hepato-und Hepatosplenomegalie, die interstitielle Pneumonie und Nephropathien. Bei den wiederholt beobachteten Hepatomegalien wurden nur vereinzelt Biopsien entnommen, ohne dass Plasmazellinfi ltrate nachgewiesen werden konnten [3,16,18,22]. Die bei der KSP beschriebene interstitielle Pneumonie zeichnet sich durch lymphozytäre und plasmazelluläre Infi ltrate aus [3,14,16,18,23].…”

Section: Extrakutane Manifestationunclassified

“…Bei den wiederholt beobachteten Hepatomegalien wurden nur vereinzelt Biopsien entnommen, ohne dass Plasmazellinfi ltrate nachgewiesen werden konnten [3,16,18,22]. Die bei der KSP beschriebene interstitielle Pneumonie zeichnet sich durch lymphozytäre und plasmazelluläre Infi ltrate aus [3,14,16,18,23]. Die interstitielle Pneumonie mit Dysproteinämie wird zur Gruppe der gutartigen Plasmazellproliferationen gezählt, so dass ein direkter Zusammenhang mit der KSP möglich erscheint [22].…”

Section: Extrakutane Manifestationunclassified

“…In weiteren Einzelberichten werden Manifestationen der KSP im Bereich der Brust, des Retroperitoneums und der Nieren beschrieben, im letzteren Fall in Form einer mesangioproliferativen Glomerulonephritis oder einer Plasmazellinfi ltration [16,22,24,25]. Die im Einzelfall auftretenden allgemeinen Symptome der KSP sind unspezifi sch und umfassen Müdigkeit, Gewichtsverlust, Fieber und Dyspnoe [3,9,23].…”

Section: Extrakutane Manifestationunclassified

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Kutane und systemische Plasmozytose

Wagner

Rosé²,

Klapper

et al. 2013

J Deutsche Derma Gesell

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SummaryCutaneous and systemic plasmacytosis is a rare disorder observed mainly in Japanese that features an infiltration of mature plasma cells in various organ systems. In addition to the skin, lymph nodes and bone marrow are regularly affected. Laboratory tests show a polyclonal hypergammaglobulinemia. The cutaneous morphology is characterized by red to dark brown macules, papules and plaques a few centimeters in diameter, usually distributed symmetrically on the face, neck and back. Etiology and pathogenesis are not known. It is speculated that a reactive dysfunction of plasma cells may be triggered by various stimuli, such as interleukin 6. Treatment of cutaneous and systemic plasmacytosis is difficult. A standardized treatment concept does not yet exist. Topical corticosteroids and calcineurin inhibitors are mainly used.

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Systemic Plasmacytosis and the Crow-Fukase Syndrome

Burton¹

1987

Arch Dermatol

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Systemic plasmacytosis. A syndrome of peculiar multiple skin eruptions, generalized lymphadenopathy, and polyclonal hypergammaglobulinemia

Cited by 47 publications

References 18 publications

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

Kutane und systemische Plasmozytose

Systemic Plasmacytosis and the Crow-Fukase Syndrome

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