Cutaneous and Systemic Plasmacytosis Showing Histopathologic Features as Mixed-Type Castleman Disease

Abstract

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder characterized by disseminated reddish brown plaques and polyclonal hypergammaglobulinemia. The lesions of CSP are histologically characterized by an infiltration of mature polyclonal plasma cells, which display similar pathological features to the plasma cell-type Castleman disease (CD). The relationship between CSP and CD is controversial. Herein, we described a 43-year-old man from China with disseminated reddish brown plaques and nodules on the cheek and temple. The serum level of immunoglobulin G and immunoglobulin A were higher than normal. In addition to mature plasma cell perivascular infiltrate in the dermis, the biopsy of the lesions showed small to medium-sized germinal follicles with hyalinized vessels and a concentrically arranged mantle zone. The patient had clinical features of CSP, but the biopsy revealed changes resembling mixed-type CD. To the best of our knowledge, this is the first case of CSP with the pathological features of mixed-type CD reported from China.

Treatment of cutaneous plasmacytosis with mask‐bath PUVA: A therapeutic option

“…As CP may pose a diagnostic challenge in the setting of other haematological malignancies such as Castleman's disease, a careful bone marrow examination is necessary. 4 Owing to the small number of patients, there has been no established definitive treatment. Reported treatment modalities of CP include intralesional corticosteroid injections, 2,5 topical or oral corticosteroids, topical chemotherapy, topical PUVA photochemotherapy, 6 radiotherapy and photodynamic therapy (PDT).…”

Kutane und systemische Plasmozytose

“…Alle 41 von Uhara et al erfassten Patienten waren Japaner [9]. Darüber hinaus sind ganz vereinzelt Chinesen, Koreaner und Thailänder betroffen gewesen [5,6,10,17,18]. Weltweit ist die KSP bis heute nur bei etwa 10 weißen Patienten beschrieben worden [12].…”

“…Histopathologisch ist die KSP von allen anderen Dermatosen abzugrenzen, die typischerweise oder gelegentlich durch Plasmazellinfi ltrationen gekennzeichnet sind. Hierzu zählen maligne monoklonale Proliferationen wie das Marginalzonenlymphom, das kutane Plasmozytom und die Leucaemia cutis bei Plasmazellleukämie [17,19,20]. Weitere histopathologische Differenzialdiagnosen mit einem polyklonalen Infi ltrationsmuster sind die B-Zell-Pseudolymphome und der multizentrische Morbus Castleman sowie bei Infektionen die kutanen Manifestationen der Syphilis und der Borreliose.…”

Cutaneous and systemic plasmocytosis

“…Histopathologically, CSP must be differentiated from other dermatoses that are typically or occasionally characterized by plasma cell infiltrations. These include malignant monoclonal proliferations, such as marginal zone lymphoma, cutaneous plasmocytoma and leukemia cutis in plasma cell leukemia [17,19,20]. Further histopathological differential diagnoses with a polyclonal infiltration pattern are the B-cell pseudolymphomas and multicentric Castleman disease as well as syphilis and borreliosis.…”

“…All 41 patients reported by Uhara et al were Japanese [9]. Further, very isolated cases have been reported in Chinese, Koreans and Thais [5,6,10,17,18]. On a worldwide basis CSP has to date only been reported in about 10 white patients [12].…”