Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text

Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates

Journal of the American Academy of Dermatology
Volume 44, n° 4
pages 593-598 (avril 2001)
Doi : 10.1067/mjd.2001.112222
accepted : 30 August 2000
Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus

Manabu Ohyama, MDa, Masayuki Amagai, MDa, Takashi Hashimoto, MDb, Hossein C. Nousari, MDc, Grant J. Anhalt, MDc, Takeji Nishikawa, MDa
Tokyo and Fukuoka, Japan, and Baltimore, Maryland 
From the Department of Dermatology, Keio University School of Medicine, Tokyoa; the Department of Dermatology, Kurume University School of Medicine, Fukuokab; and the Department of Dermatology and Pathology, Johns Hopkins University, School of Medicine, Baltimore.c 


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.)

The full text of this article is available in PDF format.

 Supported in part by Health Sciences Research Grants for Research on Specific Diseases from Ministry of Health and Welfare and a Grant-in-Aid for Scientific Research from the Ministry of Education, Science, Sports and Culture of Japan.
 Reprint requests: Masayuki Amagai, MD, Assistant Professor, Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
 J Am Acad Dermatol 2001;44:593-8

© 2001  American Academy of Dermatology, Inc. Published by Elsevier Masson SAS@@#104157@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Article Outline