Department of Dermatology, Allergology and Phlebology, Bremerhaven Reinkenheide Hospital, Bremerhaven, Germany.
J Dtsch Dermatol Ges. 2013 Dec;11(12):1161-7. doi: 10.1111/ddg.12190. Epub 2013 Aug 12.
Cutaneous 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.
皮肤和全身浆细胞瘤是一种罕见的疾病，主要发生在日本人中，其特征是成熟浆细胞浸润各种器官系统。除皮肤外，淋巴结和骨髓也经常受到影响。实验室检查显示多克隆高γ球蛋白血症。皮肤形态学表现为红色至深褐色的斑疹、丘疹和斑块，直径数厘米，通常对称分布于面部、颈部和背部。病因和发病机制尚不清楚。据推测，各种刺激物（如白细胞介素 6）可能引发浆细胞的反应性功能障碍。皮肤和全身浆细胞瘤的治疗较为困难。目前尚无标准化的治疗概念。主要使用局部皮质类固醇和钙调神经磷酸酶抑制剂。
