Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

Abstract

Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinicians.

Humoral Epitope Spreading in Autoimmune Bullous Diseases

“…Paraneoplastic pemphigus is a rare autoimmune bullous disease that is always associated with neoplasm ( 125 , 126 ). Typically, PNP patients show autoantibodies directed against the plakin family, including antibodies against envoplakin, periplakin, desmoplakins I and II, plectin, the hemidesmosomal protein BP180 and BP230 ( 125 , 126 ), and the protease inhibitor alpha-2-macroglobulin-like-1 ( 127 ). Furthermore, antibodies against plakophilin 3, desmocollins 1, desmocollins 3, Dsg1, and 3 have also been detected in PNP ( 125 , 126 ).…”

“…Typically, PNP patients show autoantibodies directed against the plakin family, including antibodies against envoplakin, periplakin, desmoplakins I and II, plectin, the hemidesmosomal protein BP180 and BP230 ( 125 , 126 ), and the protease inhibitor alpha-2-macroglobulin-like-1 ( 127 ). Furthermore, antibodies against plakophilin 3, desmocollins 1, desmocollins 3, Dsg1, and 3 have also been detected in PNP ( 125 , 126 ). Clinically, patients are characterized by the presence of severe mucocutaneous lesions and sometimes respiratory failure, and they are refractory to standard treatment.…”