Bullous pemphigoid (BP) is an acquired autoimmune subepidermal blistering disease against hemidesmosomal cytoplasmic BP230 and transmembrane BP180 proteins. Epitope mapping studies have shown that the membrane-proximal noncollagenous (NC) 16a domain of BP180 harbors clusters of antigenic sites recognized by the vast majority of BP sera. In this study, we developed an enzyme-linked immunosorbent assay (ELISA) using bacterial recombinant NC16a protein and evaluated its clinical benefit for diagnosis and monitoring disease activity. Fifty four (84.4%) of 64 sera from BP patients were positive, while only one (1.1%) of 91 sera from collagen disease patients and five (1.5%) of 336 sera from normal control barely exceeded the cut-off value. None of 69 pemphigus vulgaris sera and none of 42 pemphigus foliaceus sera exceeded the cut-off value. Thus, the sensitivity and specificity of NC16a ELISA were 84.4 and 98.9%, respectively. The correlation between ELISA scores and disease activity along the time course was examined using seven BP patients. NC16a ELISA scores tended to fluctuate in parallel with the disease activity along the time course and reflected the disease activity much better than indirect immunofluorescence. These findings indicate that NC16a ELISA will be a valuable tool not only for the diagnosis of patients with BP but also for the monitoring of the disease activity.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Journal of Dermatological Science
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Cell adhesion molecules as targets of autoantibodies in pemphigus and pemphigoid, bullous diseases due to defective epidermal cell adhesion.Adv. Immunol. 1993; 53: 291-325
- Rabbits immunized with a peptide encoded for by the 230-kDa bullous pemphigoid antigen cDNA develop an enhanced inflammatory response to UVB irradiation: a potential animal model for bullous pemphigoid.J. Invest. Dermatol. 1993; 101: 9-14
- Cloning and primary structural analysis of the bullous pemphigoid autoantigen, BP180.J. Invest. Dermatol. 1992; 99: 243-250
- Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain.J. Immunol. 1993; 151: 5742-5750
- The majority of bullous pemphigoid and herpes gestationis serum samples react with NC16a domain of the 180 kDa bullous pemphigoid antigen.Arch. Dermatol. Res. 1996; 288: 507-509
- Analysis of antigens recognized by autoantibodies in herpes gestationis: usefulness of immunoblotting using a fusion protein representing an extracellular domain of the 180 kDa bullous pemphigoid antigen.J. Dermatol. Sci. 1996; 13: 112-117
- Tight clustering of extracellular BP180 epitopes recognized by bullous pemphigoid autoantibodies.J. Invest. Dermatol. 1997; 109: 573-579
- A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180.J. Clin. Invest. 1993; 92: 2480-2488
- Bullous pemphigoid. Clinical and immunologic follow-up after successful therapy.Arch. Dermatol. 1977; 113: 1043-1046
- Lack predictive factors for the clinical course of bullous pemphigoid.J. Am. Acad. Dermatol. 1992; 26: 585-589
- A highly sensitive enzyme-linked immunosorbent assay for the detection of circulating anti-BP180 autoantibodies in patients with bullous pemphigoid.J. Invest. Dermatol. 1997; 109: 679-683
- Serum levels of autoantibodies in patients with bullous pemphigoid.Arch. Dermatol. 2000; 136: 253-254
- Clinical significance of enzyme-linked immunosorbent assay for the detection of circulating anti-BP180 autoantibodies in patients with bullous pemphigoid.J. Dermatol. Sci. 2001; 26: 14-18
- Detection of pemphigus vulgaris and pemphigus foliaceus antigens by immunoblot analysis using different antigen sources.J. Invest. Dermatol. 1990; 94: 327-331
- Development of an ELISA to detect anti-BP180 autoantibodies in bullous pemphigoid and herpes gestationis.J. Invest. Dermatol. 1994; 102: 878-881
- Immunoreactivity of bullous pemphigoid (BP) autoantibodies against the NC16A and C-terminal domains of the 180 kDa BP antigen (BP180): immunoblot analysis and enzyme-linked immunosorbent assay using BP180 recombinant proteins.Br. J. Dermatol. 1998; 139: 365-370
- Production of the entire extracellular domain of BP180 (type XVII collagen) by baculovirus expression.J. Dermatol. Sci. 2000; 23: 183-190
Accepted: August 22, 2002
Received in revised form: August 20, 2002
Received: June 27, 2002
© 2002 Elsevier Science Ireland Ltd. Published by Elsevier Inc. All rights reserved.