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Key Clinical Message  Paraneoplastic pemphigus ( PNP ) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with  PNP  and myasthenia gravis associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma, as well as the response to rituximab.

Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma: response to rituximab