Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report.

ABSTRACT: Castleman disease (CD) is a rare lymphoproliferative disorder that may be complicated by paraneoplastic pemphigus (PNP) and follicular dendritic cell sarcoma (FDCS), although their concurrence is uncommon. The present study reported on a 67-year-old woman with progressive oral ulceration and generalized purple skin spots. Imaging demonstrated a 114mm ×96mm ×118 mm lesion in the left pelvis. Serology was positive for anti-desmoplakin-1 and -3 and anti-BP230 antibodies. Histopathology revealed hyaline-vascular unicentric CD with focal low-to-intermediate-grade FDCS; immunohistochemistry showed CD21, CD23, CD35, CD68, CXCL13 expression and Ki-67 ~10%. Initial therapy comprised high-dose methylprednisolone, intravenous immunoglobulin and thalidomide, followed by surgical resection and adjuvant

SUBMITTER: Li Y 

PROVIDER: S-EPMC12690628 | biostudies-literature | 2026 Feb

REPOSITORIES: biostudies-literature