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 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.


CONCLUSIONS
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.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
M
M
Ohyama
M
M
Amagai
T
T
Hashimoto
H C
HC
Nousari
G J
GJ
Anhalt
T
T
Nishikawa
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D003598:Cytoskeletal Proteins / Q000276:immunology*; D051183:Desmoglein 1; D051182:Desmogleins; D051180:Desmoplakins; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000097:blood / Q000235:genetics / Q000276:immunology*; D010392:Pemphigus / Q000097:blood / Q000235:genetics / Q000276:immunology*
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D003598:Cytoskeletal Proteins; C495297:DSG1 protein, human; D051183:Desmoglein 1; D051182:Desmogleins; D051180:Desmoplakins