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.
Ohyama
M
M
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Amagai
M
M
Hashimoto
T
T
Nousari
H C
HC
Anhalt
G J
GJ
Nishikawa
T
T
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