The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile,☆☆,

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Abstract

Background: Some patients with pemphigus vulgaris (PV) have mucous membrane erosions with minimal skin involvement (mucosal dominant type), and others show extensive skin blisters and erosions in addition to mucous membrane involvement (mucocutaneous type). Patients with pemphigus foliaceus (PF) show only skin involvement. Objective: The purpose of this study is to determine whether there is a difference in autoantibody profile among mucosal dominant PV, mucocutaneous PV, and PF. Methods: Antibody titer against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3) were measured with enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3. Sera were obtained during clinically active disease from 24 patients with mucosal dominant PV, 20 with mucocutaneous PV, and 23 with PF. Results: All sera samples from patients with mucosal dominant PV sera were negative against Dsg1 but positive against Dsg3. All sera samples from those with mucocutaneous PV were positive against both Dsg1 and Dsg3. All sera samples from patients with PF were positive against Dsg1, but negative against Dsg3. Conclusion: Each subtype has its own anti-Dsg autoantibody profile, indicating that the clinical phenotype of pemphigus is defined by the autoantibody profile. (J Am Acad Dermatol 1999;40:167-70.)

Section snippets

Human sera

Sera were obtained from 44 patients with clinically, histologically, and immunopathologically typical PV and 23 patients with typical PF. Patients with PV were classified as having mucosal dominant type and mucocutaneous type. Mucosal dominant type showed predominant oral erosions with limited skin involvement, which was no more than 5 or 6 scattered or isolated erosions or blisters no larger than 5 cm in diameter. Mucocutaneous type showed extensive skin involvement in addition to oral

Mucosal dominant PV has only anti-Dsg3 IgG, but mucocutaneous PV has both anti-Dsg3 and anti-Dsg1 IgG

When we examined the clinical phenotype of PV, this disease could be further divided into two subgroups. One is a mucosal dominant type, which has extensive oral erosions or nasal, esophageal, or ocular erosions with limited involvement of the skin. This phenotype can have several scattered skin blisters or erosions. The other is a mucocutaneous type that has extensive skin blisters and erosions in addition to oral and other mucosal involvement. In general, the mucocutaneous type tends to be

DISCUSSION

It has been documented that some patients with PV have only anti-Dsg3 antibody, whereas other patients with PV have both anti-Dsg1 and anti-Dsg3 antibody.5, 6, 7 However, it was unclear whether there is any difference in the clinical phenotype of Dsg1(-) PV and Dsg1(+) PV patients. Previously we noted that patients with PV who have significant skin involvement tend to have higher anti-Dsg1 IgG titers than those with predominant oral lesions.11 Our previous study included some serum samples

Acknowledgements

We are grateful to Dr John R. Stanley for insightful discussions. We thank Mrs Minae Suzuki for help with the ELISA work and Dr Kazuhiko Ohya for preparing the reagents for Dsg3 and Dsg1 ELISA.

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    Pemphigus (vulgaris and foliaceus) is a debilitating disease with painful blisters of the skin and mucosal surfaces that can cause severe and fatal complications. The disease is characterized by IgG autoantibodies directed at the desmosomal cadherins desmogleins (DSGs) 1 and 3, important for keratinocyte cell adhesion in tissues subjected to mechanical stress (Amagai et al., 1999; Huijbers et al., 2018). Disease activity is associated with the levels of anti-DSG1 and anti-DSG3 autoantibodies in the circulation, and their reduction has been shown to correlate with clinical response (Joly et al., 2017; Yeoh et al., 2019).

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Supported by Grant-In-Aid for Scientific Research from the Ministry of Education, Science, and Culture of Japan, and Research Grants for Life Sciences and Medicine, Keio University Medical Science Fund, and Keio Gijuku Academic Development Funds.

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Reprint requests: Masayuki Amagai, MD, PhD, Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

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