Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus

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 mu...

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Published in:Journal of the American Academy of Dermatology 2001-04, Vol.44 (4), p.593-598
Main Authors: Ohyama, Manabu, Amagai, Masayuki, Hashimoto, Takashi, Nousari, Hossein C., Anhalt, Grant J., Nishikawa, Takeji
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Autoantibodies - blood
Biological and medical sciences
Bullous diseases of the skin
Cell Adhesion Molecules - immunology
Cytoskeletal Proteins - immunology
Dermatology
Desmoglein 1
Desmogleins
Desmoplakins
Female
Humans
Immunophenotyping
Male
Medical sciences
Middle Aged
Paraneoplastic Syndromes - blood
Paraneoplastic Syndromes - genetics
Paraneoplastic Syndromes - immunology
Pemphigus - blood
Pemphigus - genetics
Pemphigus - immunology
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Summary: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. (J Am Acad Dermatol 2001;44:593-8.)
ISSN:0190-9622
1097-6787
DOI:10.1067/mjd.2001.112222