• Chang Qu, Xiaodong Tian, Yongsu Ma, Xuehai Xie, Mingyue Wang, Yujun Dong, Jixin Zhang, Ping Liu, and Yinmo Yang.
• Department of General Surgery.
• Medicine (Baltimore). 2020 Dec 18; 99 (51): e23588.

RationaleFollicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment.Patient ConcernsA 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP.DiagnosesPNP with FDCS.InterventionsThe patient received gamma globulin infusion, took anlotinib, and underwent plasma exchange therapy.OutcomesThe skin lesions recovered and there was no evidence of tumor recurrence.LessonsThe diagnosis and management of PNP with FDCS require close cooperation among surgeons, dermatologists, hematologists, otolaryngologists, oncologists, radiologists, pathologists, and respiratory doctors. The interesting clinical manifestations of this patient provide a multifaceted approach to the investigation of the interactions among FDCS, Castleman disease, and PNP.Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.

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