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BMZ: basement membrane zone ELISA: enzyme-linked immunosorbent assay FDCS: follicular dendritic cell sarcoma IIF: indirect immunofluorescence IVIG: intravenous immunoglobulin PNP: paraneoplastic pemphigus PV: pemphigus vulgaris INTRODUCTION The association of paraneoplastic pemphigus (PNP) with malignancy is well established in the literature. PNP has been reported to occur in the setting of follicular dendritic cell sarcoma (FDCS). This neoplasm has been associated with several other autoimmune disorders, likely because of the role follicular dendritic cells play in facilitating humoral immunity. Pemphigus vulgaris (PV) may clinically resemble PNP, and a comprehensive evaluation may be necessary to differentiate these uncommon conditions in the setting of malignancy. Here we present a patient with significant, treatmentrefractory mucositis, myasthenia gravis, and a history of PV in the setting of FDCS of the thymus.

jaad case reports

Refractory mucositis associated with underlying follicular dendritic cell sarcoma of the thymus: Paraneoplastic pemphigus versus malignancy-exacerbated pemphigus vulgaris