title: 230-kDa and 190-kDa proteins in addition to desmoglein 1 as immunological targets in a subset of pemphigus foliaceus with a combined cell-surface and basement membrane zone immune staining pattern.
abstract: Pemphigus erythematosus, initially described as a combination of pemphigus with lupus erythematosus, and pemphigus foliaceus are now frequently considered localized and generalized variants of superficial pemphigus. Yet diagnostic criteria for pemphigus erythematosus remain controversial. Distinct from pemphigus foliaceus, pemphigus erythematosus displays immune depositions at the dermal-epidermal junction, which suggests additional immunopathological mechanisms. We present three patients with clinical and histopathologic signs of superficial pemphigus, who all exhibited an immunomorphology characteristic of pemphigus erythematosus. Complement depositions in a granular-linear fashion were consistently found at the dermal-epidermal junction besides in vivo bound and circulating antikeratinocyte cell-surface autoantibodies. Histopathology showed subcorneal acantholysis, and all sera contained antidesmoglein 1 but not antidesmoglein 3 autoantibodies detected by enzyme-linked immunosorbent assays (ELISA). Additional autoantibodies against a 230-kDa protein and against a 190-kDa protein comigrating with bullous pemphigoid antigen 1 (BP230) and periplakin, respectively, were present in all the patients' sera. As two sera specifically reacted with BP230 by ELISA, the presence of BP230-specific autoantibodies could be associated with dermal-epidermal immune staining in these patients. In pemphigus erythematosus, dermal-epidermal immune staining is generally attributed to the deposition of immune complexes, while the presence of BP230-specific autoantibodies has not been reported in this disease previously. Perhaps, the unique autoantibody profile of the patients in the study permits discrimination between patients with superficial pemphigus that display additional dermal-epidermal immune staining from those with conventional pemphigus foliaceus on a molecular basis. Further studies will be required to substantiate the frequency of this occurrence and to unravel its pathogenic significance.
authors.affiliation: Division of Immunology, Allergy and Infectious Diseases, Department of Dermatology, University of Vienna Medical School, General Hospital, Vienna, Austria. Franz.Karlhofer@akh-wien.ac.at
authors.forename: F M
authors.initials: FM
authors.lastname: Karlhofer
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: K
authors.initials: K
authors.lastname: Slupetzky
authors.forename: M
authors.initials: M
authors.lastname: Kiss
authors.forename: Y
authors.initials: Y
authors.lastname: Liu
authors.forename: M
authors.initials: M
authors.lastname: Amagai
authors.forename: F
authors.initials: F
authors.lastname: Pieczkowski
authors.forename: D
authors.initials: D
authors.lastname: Foedinger
authors.forename: R
authors.initials: R
authors.lastname: Kirnbauer
authors.forename: G
authors.initials: G
authors.lastname: Stingl
mesh_terms: D000328:Adult; D000954:Antigens, Surface / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology*; D001485:Basement Membrane / Q000276:immunology; D015820:Cadherins / Q000276:immunology*; D002352:Carrier Proteins / Q000276:immunology*; D003598:Cytoskeletal Proteins / Q000276:immunology*; D051183:Desmoglein 1; D000071821:Dystonin; D004797:Enzyme-Linked Immunosorbent Assay; D004817:Epidermis / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000097:blood; D009419:Nerve Tissue Proteins / Q000276:immunology*; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology; D051179:Plakins
chemical_list: D000954:Antigens, Surface; D001323:Autoantibodies; D001324:Autoantigens; D015820:Cadherins; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; C486268:DST protein, human; D051183:Desmoglein 1; D000071821:Dystonin; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; C491674:PPL protein, human; D051179:Plakins