A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading
| Autor: | Mei Chen, Janet A. Fairley, David T. Woodley, Mong-Shang Lin, George J. Giudice |
|---|---|
| Rok vydání: | 2004 |
| Předmět: |
Epidermolysis bullosa acquisita
Pathology medicine.medical_specialty Pemphigoid Collagen Type VII Immunoblotting Enzyme-Linked Immunosorbent Assay Dermatology Epidermolysis Bullosa Acquisita Autoantigens Subclass Epitope Epitopes Sequence Analysis Protein Pemphigoid Bullous Humans Medicine skin and connective tissue diseases Autoantibodies Autoimmune disease integumentary system business.industry Autoantibody Non-Fibrillar Collagens medicine.disease Phenotype Immunoglobulin G Immunology Epidermolysis bullosa Bullous pemphigoid business Follow-Up Studies |
| Zdroj: | Journal of the American Academy of Dermatology. 51:118-122 |
| ISSN: | 0190-9622 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect