title: Antibody titers to desmogleins 1 and 3 in a patient with paraneoplastic pemphigus associated with follicular dendritic cell sarcoma.
abstract: BACKGROUND
  Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. Patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role in the initial stage of PNP.
  
  
  METHODS
  We describe a patient with PNP associated with follicular dendritic cell sarcoma. Antibodies to envoplakin and periplakin were detected. When only mucosal lesions were observed at the early stage, the antibody to Dsg3 but not to Dsg1 was detected by enzyme-linked immunosorbent assay. After skin lesions appeared, antibodies to Dsg1 and Dsg3 were detected. These titers were elevated, with exacerbation of skin lesions. Although the patient received corticosteroid therapy, double-filtration plasmapheresis, and intravenous human immunoglobulin therapy after surgical resection of follicular dendritic cell sarcoma, she died of fungal infective lung embolisms. A direct immunofluorescence study of autopsy samples showed IgG deposition in the epidermis of the skin and oral mucosal membrane, but not in the lungs and kidneys and follicular dendritic cell sarcoma of the para-aortic area.
  
  
  CONCLUSIONS
  In this patient with PNP and follicular dendritic cell sarcoma, there was an association between the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.
authors.affiliation: Department of Dermatology, Ogaki Municipal Hospital, Ogaki, Japan. tosyo1@omh.ogaki.gifu.jp
authors.forename: Mariko
authors.initials: M
authors.lastname: Seishima
authors.forename: Makiko
authors.initials: M
authors.lastname: Oda
authors.forename: Zuiei
authors.initials: Z
authors.lastname: Oyama
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Yoshimura
authors.forename: Futoshi
authors.initials: F
authors.lastname: Yamazaki
authors.forename: Takahiko
authors.initials: T
authors.lastname: Aoki
authors.forename: Mariko
authors.initials: M
authors.lastname: Nei
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D000906:Antibodies / Q000097:blood*; D015820:Cadherins / Q000276:immunology*; D020566:Dendritic Cells, Follicular / Q000473:pathology; D051183:Desmoglein 1; D051184:Desmoglein 3; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D010392:Pemphigus / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D012186:Retroperitoneal Neoplasms / Q000150:complications* / Q000473:pathology; D012509:Sarcoma / Q000150:complications* / Q000473:pathology
chemical_list: D000906:Antibodies; D015820:Cadherins; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3