Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus

Manabu Ohyama, Masayuki Amagai, Takashi Hashimoto, Hossein C. Nousari, Grant J. Anhalt, Takeji Nishikawa

Research output: Contribution to journalArticle

43 Citations (Scopus)

Abstract

Background: Paraneoplastic pemphigus (PNP) has similar features to pemphigus vulgaris (PV), including circulating anti-desmoglein (Dsg) IgG as pathogenic autoantibodies. When PV is divided into mucosal dominant type and mucocutaneous type, mucosal dominant type has only anti-Dsg3 IgG, whereas the mucocutaneous type has both anti-Dsg3 and anti-Dsg1 IgG. Objective: The purpose of this study was to determine whether there is a difference in anti-Dsg autoantibody profile between mucosal dominant PNP and mucocutaneous PNP. Methods: Twenty-one patients with PNP were categorized as mucosal dominant and mucocutaneous types based on clinical information. Antibody titers against Dsg3 and Dsg1 were measured by enzyme-linked immunosorbent assay by means of recombinant Dsg1 and Dsg3. Results: There were 9 cases of mucosal dominant type and 12 cases of mucocutaneous type. Eight of 9 cases of mucosal dominant type were positive for anti-Dsg3 IgG, but 3 of them were also positive for anti-Dsg1 IgG. All 12 cases of mucocutaneous type were positive for anti-Dsg3 IgG, whereas only 6 of them were positive for anti-Dsg1 IgG. Conclusion: There was no clear association between the clinical phenotype and anti-Dsg antibody profile in PNP as seen in PV. This finding suggests that besides anti-Dsg IgG other pathologic mechanisms such as lichenoid reaction or interface dermatitis may be involved in the blister formation in PNP.

Original languageEnglish
Pages (from-to)593-598
Number of pages6
JournalJournal of the American Academy of Dermatology
Volume44
Issue number4
DOIs
Publication statusPublished - 2001

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Desmogleins
Pemphigus
Autoantibodies
Phenotype
anti-IgG
Dermatitis
Blister
Anti-Idiotypic Antibodies
Enzyme-Linked Immunosorbent Assay

ASJC Scopus subject areas

  • Dermatology

Cite this

Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus. / Ohyama, Manabu; Amagai, Masayuki; Hashimoto, Takashi; Nousari, Hossein C.; Anhalt, Grant J.; Nishikawa, Takeji.

In: Journal of the American Academy of Dermatology, Vol. 44, No. 4, 2001, p. 593-598.

Research output: Contribution to journalArticle

Ohyama, Manabu ; Amagai, Masayuki ; Hashimoto, Takashi ; Nousari, Hossein C. ; Anhalt, Grant J. ; Nishikawa, Takeji. / Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus. In: Journal of the American Academy of Dermatology. 2001 ; Vol. 44, No. 4. pp. 593-598.
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abstract = "Background: Paraneoplastic pemphigus (PNP) has similar features to pemphigus vulgaris (PV), including circulating anti-desmoglein (Dsg) IgG as pathogenic autoantibodies. When PV is divided into mucosal dominant type and mucocutaneous type, mucosal dominant type has only anti-Dsg3 IgG, whereas the mucocutaneous type has both anti-Dsg3 and anti-Dsg1 IgG. Objective: The purpose of this study was to determine whether there is a difference in anti-Dsg autoantibody profile between mucosal dominant PNP and mucocutaneous PNP. Methods: Twenty-one patients with PNP were categorized as mucosal dominant and mucocutaneous types based on clinical information. Antibody titers against Dsg3 and Dsg1 were measured by enzyme-linked immunosorbent assay by means of recombinant Dsg1 and Dsg3. Results: There were 9 cases of mucosal dominant type and 12 cases of mucocutaneous type. Eight of 9 cases of mucosal dominant type were positive for anti-Dsg3 IgG, but 3 of them were also positive for anti-Dsg1 IgG. All 12 cases of mucocutaneous type were positive for anti-Dsg3 IgG, whereas only 6 of them were positive for anti-Dsg1 IgG. Conclusion: There was no clear association between the clinical phenotype and anti-Dsg antibody profile in PNP as seen in PV. This finding suggests that besides anti-Dsg IgG other pathologic mechanisms such as lichenoid reaction or interface dermatitis may be involved in the blister formation in PNP.",
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