The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels.

Abstract

BACKGROUND:

Pemphigus vulgaris (PV) and foliaceus (PF) are characterized by antibodies to the desmosomal proteins desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1), respectively. Past studies using indirect immunofluorescence (IIF) as a measure of pemphigus antibody levels have failed to demonstrate consistently a relationship between disease severity and IIF titres. However, IIF is not able to measure separately Dsg1 and 3 antibodies, unlike enzyme-linked immunosorbent assays (ELISA), which utilize recombinant proteins.

OBJECTIVES:

To compare independently Dsg1 and 3 antibody levels with the severity of both cutaneous and oral involvement in PV and PF. Patients and methods Four hundred and twenty-four serum samples were analysed from 80 subjects with PV and 24 with PF. IgG antibodies to Dsg1 and 3 were measured by ELISA. For every sample analysed, the associated severity of skin and oral disease were graded from 0 to 3; quiescent, mild, moderate and severe.

RESULTS:

A relationship between Dsg1 antibodies and skin severity was demonstrated such that a 10-unit increase in Dsg1 ELISA value was associated with a 34% chance of having a higher severity score [95% confidence interval (CI), 25-45%, P < 0.0005]. This was observed in both PV and PF. Oral severity was associated with Dsg3 antibody levels and a 10-unit increase in the Dsg3 ELISA value was associated with a 25% chance of a higher oral severity score (CI 17-33%, P < 0.0005). We were unable to demonstrate a relationship between Dsg1 antibodies and oral severity, even after adjusting for the effect of Dsg3 antibodies. Similarly, there was no relationship between Dsg3 antibodies and skin severity.

CONCLUSIONS:

This study suggests that the clinical phenotype of pemphigus, in particular the balance of skin and oral disease, is determined principally by the quantities of Dsg1 and 3 autoantibodies, respectively.

• Authors: K E Harman, P T Seed, M J Gratian, B S Bhogal, S J Challacombe, M M Black
• Journal: The British journal of dermatology (Br J Dermatol) Vol. 144 Issue 4 Pg. 775-80 (Apr 2001) ISSN: 0007-0963 [Print] England
• PMID: 11298536 (Publication Type: Journal Article)

Chemical References

• Autoantibodies
• Autoantigens
• Biomarkers
• Cadherins
• DSG3 protein, human
• Desmoglein 1
• Desmoglein 3

Topics

• Autoantibodies (blood)
• Autoantigens (immunology)
• Biomarkers (blood)
• Cadherins (immunology)
• Desmoglein 1
• Desmoglein 3
• Enzyme-Linked Immunosorbent Assay
• Humans
• Mouth Diseases (immunology)
• Pemphigus (immunology)
• Severity of Illness Index