Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus

• 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
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1067/mjd.2001.112222

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.)