Paraneoplastic Autoimmune Multi-organ Syndrome: Association with Retroperitoneal Kaposi's Sarcoma

© 2015 The Authors. doi: 10.2340/00015555-2169 Journal Compilation © 2015 Acta Dermato-Venereologica. ISSN 0001-5555 Paraneoplastic pemphigus (PNP), described in 1990 by Anhalt et al. (1), is an autoimmune mucocutaneous blistering disease characterized by severe mucosal erosions and polymorphic cutaneous lesions. In 2001 the term paraneoplastic autoimmune multi-organ syndrome (PAMS) was coined (2) to reflect its frequent multi-organ involvement and numerous clinical variants (pemphiguslike, pemphigoid-like, erythema multiforme-like, graftversus-host disease-like, and lichen planus-like). This disease is most often associated with haematological malignancies, and has a high mortality rate (90%) with less than one-year mean survival. We report here a case of PNP/PAMS that was associated with retroperitoneal Kaposi’s sarcoma (KS) and evolved through 3 different clinical variants (pemphigus-like, lichen planus-like and erythema multiformelike). This is the first report of an association of PNP/ PAMS with KS in literature.