Anhalt et al. [7] in 1990, suggested the name paraneoplastic pemphigus for a pemphigus variant with underlying neoplasms wherein painful mucosal ulcerations and polymorphous skin lesions develop and is characterized by pathogenic autoantibodies directed against desmoplakin-I, BPAG-I, envoplakin-I, and periplakin. This evidence concerns the gene PPL and paraneoplastic pemphigus.