Paraneoplastic pemphigus with anti-desmocollin 3 autoantibodies and chronic lymphocytic leukemia

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disorder coexisting with certain neoplasms, most commonly non-Hodgkin lymphoma, chronic lymphocytic leukemia (CLL) and Castleman’s disease [1]. The clinical presentation of PNP consists of painful mucosal erosions, polymorphic cutaneous lesions and systemic involvement. The PNP is characterized by various autoantibodies directed mainly to desmosomal proteins, particularly various plakin family proteins [2]. Recently the 170 kDa α2-macroglobulin-like protein 1 was identified as another PNP antigen [3]. In addition, novel ELISA of mammalian recombinant proteins (RP)s detected autoantibodies to desmocollins 1 (Dsc1) to Dsc3 in 67% of PNP cases [4]. Herein, we report an unusual case of PNP associated with CLL, mediated by antibodies against Dsc3