Periplakin and envoplakin are target antigens in canine and human paraneoplastic pemphigus

Binding of protein kinase B to the plakin family member periplakin

“…Periplakin is expressed in epithelial cells (Aho et al, 1998), where it is found to be, together with envoplakin, the precursor of the epidermal cornified envelope (DiColandrea et al, 2000). Furthermore, periplakin has been shown to be a target antigen in paraneoplastic pemphigus (de Bruin et al, 1999), and in keratinocytes it localizes at desmosomes and the interdesmosomal plasma membrane (Simon and Green, 1984). Although these observations suggest a specified role of plakin family members in epidermal cornification there is substantial evidence that within other cell types plakins may be involved in a variety of cellular processes.…”

Progressive Respiratory Failure in Paraneoplastic Pemphigus Associated with Chronic Lymphocytic Leukemia

“…hepatocytes). Autoantibodies against plakin proteins might be internalized after binding to a transmembrane cell surface protein such as the 170-kD protein [9]. This could explain the involvement of additional organs besides skin in paraneoplastic pemphigus.…”

“…In the first description, 5 criteria were cited for the diagnosis of this entity: (1) mucosal lesions (mainly recalcitrant erosive stomatitis) and polymorphic skin eruptions (erythema-multiforme-like, lichenoid, bullous, erosive); (2) acantholysis, dyskeratosis as well as lichenoid and vacuolar interface changes in histology; (3) intraepidermal and granular or linear subepidermal deposition of IgG and C3 in direct immunofluorescence; (4) circulating autoantibodies reacting with human skin and with transitional epithelium such as urinary bladder; (5) serum immunoprecipitation of a characteristic complex of high-molecular-weight proteins of 250, 230, 210, 190 and 170 kD, corresponding to desmoplakins I [2]and II [6], BPAG-1 [2], envoplakin [9], periplakin [9]and a still unidentified 170-kD protein.…”

Paraneoplastic Pemphigus in a Patient with a Thymoma

“…These protein antigens were identified as desmoplakin I [2]and II [18], envoplakin [16], the bullous pemphigoid antigen I [2]and periplakin [16]. Desmoplakins are also present in the respiratory epithelium.…”

“…Subsequently, the autoantibodies of the plakin family might penetrate the cell and cause further damage. Another possibility is that the antibodies are internalized after binding a transmembrane cell surface protein such as the 170-kD protein [16]. …”