Paraneoplastic pemphigus (PNP) is an autoimmune blisterimng disease, which presents Stevens-Johnson syndrome-like mucocutaneous lesions and graft-ver- sus-host disease-like histopathological findings and is associated most commonly with lymphoproliferative neoplasms

© 2014 The Authors. doi: 10.2340/00015555-1742 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Paraneoplastic pemphigus (PNP) is an autoimmune blisterimng disease, which presents Stevens-Johnson syndrome-like mucocutaneous lesions and graft-versus-host disease-like histopathological findings and is associated most commonly with lymphoproliferative neoplasms (1–3). Various plakin family proteins are the major autoantigens, by which the diagnosis of PNP can be made with a high sensitivity and specificity (4). However, PNP cases without detectable malignancy have also been reported (5–7). In addition, PNP is now known to show distinct features from classical pemphigus, including nonacantholytic histological changes (3), pathogenic role of cellular immunity (8) and involvement of extracutaneous organs (7, 9, 10). Particularly, PNP-related bronchiolitis obliterans (BO) is always lethal (7, 10). We present a patient with clinically and immunologically typical PNP, who showed several unique findings and distinct efficacy of plasma exchange (PE) therapy.