A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading

Janet A Fairley; David T Woodley; Mei Chen; George J Giudice; Mong-Shang Lin

doi:10.1016/j.jaad.2003.12.033

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A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading

Journal article

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A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading

Janet A Fairley, David T Woodley, Mei Chen, George J Giudice and Mong-Shang Lin

Journal of the American Academy of Dermatology, Vol.51(1), pp.118-122

07/2004

DOI: 10.1016/j.jaad.2003.12.033

PMID: 15243536

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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.

Epidermolysis Bullosa Acquisita - immunology

Enzyme-Linked Immunosorbent Assay

Follow-Up Studies

Immunoglobulin G - blood

Autoantibodies - blood

Humans

Collagen Type VII - immunology

Immunoblotting

Epitopes

Non-Fibrillar Collagens - immunology

Sequence Analysis, Protein

Phenotype

Immunoglobulin G - genetics

Autoantigens - immunology

Autoantibodies - genetics

Pemphigoid, Bullous - immunology

Details

Title: Subtitle: A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading
Creators: Janet A Fairley - Department of Dermatology, Medical College of Wisconsin, Milwaukee 53226, USA. jfairley@mcw.edu
David T Woodley
Mei Chen
George J Giudice
Mong-Shang Lin
Resource Type: Journal article
Publication Details: Journal of the American Academy of Dermatology, Vol.51(1), pp.118-122
DOI: 10.1016/j.jaad.2003.12.033
PMID: 15243536
NLM abbreviation: J Am Acad Dermatol
ISSN: 0190-9622
eISSN: 1097-6787
Publisher: Elsevier Inc; United States
Grant note: R01 AI48427 / NIAID NIH HHS R01 AR40410 / NIAMS NIH HHS R01 AR47981 / NIAMS NIH HHS R01 AR33625 / NIAMS NIH HHS
Language: English
Date published: 07/2004
Academic Unit: Dermatology
Record Identifier: 9984025332702771

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