Systemic and cutaneous plasmacytosis with multiple skin lesions and polyclonal hypergammaglobulinaemia: significant serum interleukin-6 levels

Abstract

We report two patients who developed benign plasmacytosis with multiple skin lesions. The cases were characterized by hyperplasia of mature plasma cells, and polyclonal hypergammaglobulinaemia. One patient had hyperplasia of mature plasma cells not only in the skin, but also extensively in lymph nodes and the retroperitoneal areas around the ureters. The other had plasma cell hyperplasia limited to the skin. Extensive investigations failed to reveal any clinical or laboratory evidence suggesting the presence of any underlying disease accompanying the hypergammaglobulinaemia and/or plasma cell proliferation, such as chronic infectious disease, collagen disease or other chronic inflammatory disorder. Clinically and histologically, the first patient showed features compatible with a diagnosis of systemic plasmacytosis and the second with a diagnosis of cutaneous plasmacytosis. Significant serum interleukin-6 (IL-6) levels were detected in both patients, suggesting that IL-6 may be involved in the pathogenesis of these conditions.

Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

“…Some patients with CSP sometimes have interstitial pneumonia. Only three cases with CSP have been reported to be complicated with nephro-urological disease, one case developed interstitial nephritis, and two cases showed thickening of both ureters [3,7,8]. To the best of our knowledge, this is the first case of glomerular disease accompanied by CSP.…”

Kutane und systemische Plasmozytose

“…Die interstitielle Pneumonie mit Dysproteinämie wird zur Gruppe der gutartigen Plasmazellproliferationen gezählt, so dass ein direkter Zusammenhang mit der KSP möglich erscheint [22]. 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].…”

“…Für den multizentrischen Morbus Castleman typisch ist eine erhöhte Serumkonzentration von IL-6, einem Zytokin der Plasmazelldifferenzierung, und der Nachweis des humanen Herpesvirus Typ 8 (HHV-8) [25,31]. Während eine IL-6-Erhöhung auch bei der KSP regelmäßig nachgewiesen werden konnte, war das Virus hier nicht detektierbar [7,20,30,32].…”

Cutaneous and systemic plasmocytosis

“…The interstitial pneumonia with dysproteinemia is classified in the group of benign plasma cell proliferations, so that a direct connection with CSP appears possible [22]. In further individual cases, manifestations of CSP in the breast, the retroperitoneum and the kidneys have been described, in the latter case in the form of a mesangioproliferative glomerulonephritis or a plasma cell infiltration [16,22,24,25].…”

“…On the basis of comparable clinical and laboratory chemical findings, some authors suspect that CSP is a variant of multicentric Castleman disease, in which a plasma cell-rich lymphadenopathy, hepatosplenomegaly, disturbances of general well-being and hypergammaglobulinemia may be present [27][28][29][30][31]. Typical for multicentric Castleman disease is an elevated serum concentration of IL-6, a cytokine of plasma cell differentiation, and the detection of human herpes virus type 8 (HHV-8) [25,31]. While an elevation of IL-6 is usually found in CSP, HHV-8 has not been detected , so it appears unlikely as a possible cause of CSP [7,20,30,32].…”