Department of Dermatology, Baylor Scott & White/Texas A&M, Temple, TX.
Department of Dermatology, Oregon Health & Science University, Portland, OR; and.
Am J Dermatopathol. 2022 Apr 1;44(4):294-296. doi: 10.1097/DAD.0000000000002109.
Cutaneous/systemic plasmacytosis (C/SP) is a plasma cell disorder characterized by reddish-brown patches, lymphadenopathy, and hypergammaglobulinemia. The degree to which C/SP overlaps with other plasma cell proliferative disorders and neoplasms is incompletely understood. We present the case of a patient with a several-year history of cutaneous plasmacytosis and evidence of systemic involvement with concurrent idiopathic multicentric Castleman disease (iMCD) involving a lymph node. There have been only a few reports of systemic iMCD preceded by a long, asymptomatic phase of cutaneous manifestations. We discuss the relationship between C/SP and iMCD and elaborate on the pathophysiological overlap of these 2 conditions and potential similarities in their pathogenesis. We suggest that the 2 diseases may represent the same entity presenting on a spectrum, with individuals diagnosed with C/SP at risk for progression to iMCD.
皮肤/全身浆细胞瘤（C/SP）是一种浆细胞疾病，其特征为红棕色斑块、淋巴结病和高丙种球蛋白血症。C/SP 与其他浆细胞增殖性疾病和肿瘤重叠的程度尚不完全清楚。我们报告了一例患有皮肤浆细胞瘤数年病史且有全身受累证据的患者，同时伴有并发的特发性多中心 Castleman 病（iMCD）累及淋巴结。仅有少数报道称全身性 iMCD 之前有长时间无症状的皮肤表现阶段。我们讨论了 C/SP 和 iMCD 之间的关系，并详细阐述了这两种疾病的病理生理学重叠以及它们发病机制中的潜在相似性。我们建议这两种疾病可能代表同一实体在谱上的表现，诊断为 C/SP 的个体有进展为 iMCD 的风险。
