title: A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia.
abstract: Paraneoplastic pemphigus is an autoimmune vesiculobullous and erosive mucocutaneous disease associated with an underlying malignancy. Reported malignancies include chronic lymphocytic leukemia, non-Hodgkin's lymphoma, Castleman's disease, sarcomas, and rarely solid tumors. Patients with paraneoplastic pemphigus develop characteristic IgG autoantibodies against several antigens including members of the plakin family, bullous pemphigoid antigen 1, and desmosomal proteins. IgA pemphigus is another recently characterized immunobullous disease that presents as a vesiculopustular eruption with neutrophilic infiltration and epidermal acantholysis. Mucous membrane involvement is rare. We report what is to our knowledge a unique case with features of both IgA pemphigus and paraneoplastic pemphigus associated with chronic lymphocytic leukemia.
authors: Department of Dermatology, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
authors: Adam R
authors: AR
authors: Taintor
authors: Kristin M
authors: KM
authors: Leiferman
authors: Takashi
authors: T
authors: Hashimoto
authors: Norito
authors: N
authors: Ishii
authors: John J
authors: JJ
authors: Zone
authors: Christopher M
authors: CM
authors: Hull
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism*; D007155:Immunologic Factors / Q000627:therapeutic use; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000188:drug therapy; D008297:Male; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000276:immunology* / Q000473:pathology; D010392:Pemphigus / Q000150:complications* / Q000188:drug therapy; D000069283:Rituximab; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007070:Immunoglobulin A; D007155:Immunologic Factors; D000069283:Rituximab; D014740:Vidarabine; C024352:fludarabine