Cutaneous plasmacytosis: Report of 6 cases with or without systemic involvement
Cutaneous plasmacytosis: Report of 6 cases with or without systemic involvement
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DOI：
10.1016/j.jaad.2012.11.031
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发表时间：
2013-06-01
影响因子：
13.8
通讯作者：
Ishiko, Akira
中科院分区：
文献类型：
--
作者：
Honda, Rie;Cerroni, Lorenzo;Ishiko, Akira
背景：皮肤浆细胞增多症和系统性浆细胞增多症是罕见的实体，主要发生在日本血统的患者。目的：探讨皮肤和全身性浆细胞增多症的临床病理特征。方法：我们描述了6例皮肤浆细胞增多症患者的临床病理特征（男：女= 3：1;平均年龄：49.7岁;中位年龄：50.5岁;年龄范围：29-64岁）。患者临床表现为多发性红棕色浸润斑块和扁平肿瘤，主要位于躯干。组织学检查显示，在所有情况下，成熟的浆细胞群的真皮内的存在。3例可见具有反应性生发中心的类滤泡。5例浆细胞免疫球蛋白轻链呈预期的多克隆表达，但1例为单克隆表达，与皮肤边缘区淋巴瘤表现出重叠特征。一名患者发现重叠的功能与多中心Castleman diseases.Limitations：这是一个回顾性研究相对较少的patients.Conclusions：尽管典型的临床表现，我们的一些患者提出的组织病理学和免疫组化特征，偏离传统的外观。我们的观察证实并扩展了以前对这种难以捉摸的实体的观察，表明临床病理表现的范围可能比以前认识到的更广。
Background: Cutaneous plasmacytosis and systemic plasmacytosis are rare entities arising primarily in patients of Japanese descent. The origin and exact pathogenesis are poorly understood.Objective: We sought to determine clinicopathologic features of cutaneous and systemic plasmacytosis.Methods: We describe the clinicopathologic features of 6 patients with cutaneous plasmacytosis with or without systemic involvement (male: female = 3: 1; mean age: 49.7 years; median age: 50.5 years; age range: 29-64 years).Results: Patients presented clinically with multiple, red-brown infiltrated plaques and flat tumors, mainly located on the trunk. Histology revealed in all cases the presence of clusters of mature plasma cells within the dermis. Lymphoid follicles with reactive germinal centers were seen in 3 cases. Five cases showed the expected polyclonal expression of immunoglobulin light chain by the plasma cells, but monoclonality was observed in 1 case, thus showing overlapping features with cutaneous marginal zone lymphoma. One patient revealed overlapping features with multicentric Castleman disease.Limitations: This was a retrospective study on a relatively small number of patients.Conclusions: Despite typical clinical presentation, some of our patients presented with histopathologic and immunohistochemical features that deviated from the conventional appearance. Our observation confirms and expands previous observations of this elusive entity, suggesting that the spectrum of clinicopathologic presentations may be wider than previously recognized.