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A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading.
J Am Acad Dermatol. 2004 Jul; 51(1):118-22.JA

Abstract

Bullous pemphigoid (BP) and epidermolysis bullosa acquisita are distinct autoimmune blistering disorders. BP is characterized by autoantibodies directed against the NC16A domain of collagen XVII, whereas patients with epidermolysis bullosa acquisita have autoantibodies against the NC1 domain of type VII collagen. We followed up a patient with BP for 9 years. During that time his clinical disease took on several features suggestive of epidermolysis bullosa acquisita. The objective of this study was to determine if the patient's autoantibody profile reflected the change in his clinical picture. Enzyme-linked immunosorbent assay and immunoblotting for detection and subclass determination of autoantibodies to type XVII and type VII collagen were performed on banked patient sera from the 9-year period. The patient's initial autoantibodies were exclusively IgG1 directed against collagen XVII. During the course of his illness, the subclass specificity of the patient's type XVII collagen autoantibodies shifted to the IgG4 subclass and during the same time interval the patient developed IgG2 autoantibodies directed against type VII collagen. This patient with BP exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in the evolution of his autoimmune disease.

Authors+Show Affiliations

Department of Dermatology, Medical College of Wisconsin, Milwaukee 53226, USA. jfairley@mcw.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

15243536

Citation

Fairley, Janet A., et al. "A Patient With Both Bullous Pemphigoid and Epidermolysis Bullosa Acquisita: an Example of Intermolecular Epitope Spreading." Journal of the American Academy of Dermatology, vol. 51, no. 1, 2004, pp. 118-22.
Fairley JA, Woodley DT, Chen M, et al. A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading. J Am Acad Dermatol. 2004;51(1):118-22.
Fairley, J. A., Woodley, D. T., Chen, M., Giudice, G. J., & Lin, M. S. (2004). A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading. Journal of the American Academy of Dermatology, 51(1), 118-22.
Fairley JA, et al. A Patient With Both Bullous Pemphigoid and Epidermolysis Bullosa Acquisita: an Example of Intermolecular Epitope Spreading. J Am Acad Dermatol. 2004;51(1):118-22. PubMed PMID: 15243536.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading. AU - Fairley,Janet A, AU - Woodley,David T, AU - Chen,Mei, AU - Giudice,George J, AU - Lin,Mong-Shang, PY - 2004/7/10/pubmed PY - 2005/3/23/medline PY - 2004/7/10/entrez SP - 118 EP - 22 JF - Journal of the American Academy of Dermatology JO - J Am Acad Dermatol VL - 51 IS - 1 N2 - Bullous pemphigoid (BP) and epidermolysis bullosa acquisita are distinct autoimmune blistering disorders. BP is characterized by autoantibodies directed against the NC16A domain of collagen XVII, whereas patients with epidermolysis bullosa acquisita have autoantibodies against the NC1 domain of type VII collagen. We followed up a patient with BP for 9 years. During that time his clinical disease took on several features suggestive of epidermolysis bullosa acquisita. The objective of this study was to determine if the patient's autoantibody profile reflected the change in his clinical picture. Enzyme-linked immunosorbent assay and immunoblotting for detection and subclass determination of autoantibodies to type XVII and type VII collagen were performed on banked patient sera from the 9-year period. The patient's initial autoantibodies were exclusively IgG1 directed against collagen XVII. During the course of his illness, the subclass specificity of the patient's type XVII collagen autoantibodies shifted to the IgG4 subclass and during the same time interval the patient developed IgG2 autoantibodies directed against type VII collagen. This patient with BP exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in the evolution of his autoimmune disease. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/15243536/A_patient_with_both_bullous_pemphigoid_and_epidermolysis_bullosa_acquisita:_an_example_of_intermolecular_epitope_spreading_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190962204005316 DB - PRIME DP - Unbound Medicine ER -