A patient with both bullous pemphigoid and epidermolysis bullosa acquisita: an example of intermolecular epitope spreading - 24/08/11
Milwaukee, Wisconsin, and Los Angeles, California
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.
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Supported in part by a Merit Review Award from the Department of Veterans Affairs (Dr Fairley) and grants from the National Institutes of Health: RO1 AR33625 (Dr Woodley), RO1 AR47981 (Dr Chen), RO1 AR40410 (Dr Giudice), and RO1 AI48427 (Dr Lin). Conflicts of interest: None identified. |
Vol 51 - N° 1
P. 118-122 - juillet 2004 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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