Paraneoplastic pemphigus (PNP) is a rare and autoimmune mucocutaneous disease associated with underlying neoplasia. The diagnosis of PNP is often difficult since skin manifestations maybe initially similar to other dermatological disorders. The case is here reported of an 84-year old male diagnosed with PNP associated with CLL, who initially presented with severe diffuse erythematous lesions in the oral mucosa and nasal mucosa, and vesicular-bullous lesions on the trunk and extremities. The patient died due to respiratory failure and hypotension caused by acute PTE.

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