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

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 thalidomide-cyclophosphamide-prednisone. Postoperatively, inflammatory indices normalized and mucocutaneous lesions partially improved; nevertheless, the patient died of respiratory failure 6 months after surgery. In summary, the present case report aimed to provide valuable references and experiences for clinicians in the diagnosis and treatment of CD featuring PNP and FDCS.