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Bullous and cicatricial pemphigoid
1Johns Hopkins University, Department of Dermatology, Baltimore, Maryland 21205.
Journal of Autoimmunity
|February 1, 1991
Summary
Bullous pemphigoid (BP) is an autoimmune blistering disease. Autoantibodies target hemidesmosome proteins, causing skin blistering, though definitive proof of this mechanism is still incomplete.
Area of Science:
- Dermatology
- Immunology
- Cell Biology
Background:
- Bullous pemphigoid (BP) and cicatricial pemphigoid are autoimmune blistering diseases.
- These conditions involve epithelial detachment from the stroma, with IgG and complement deposition at the lamina lucida.
- The primary immune response involves IgG4 subclass autoantibodies targeting hemidesmosome proteins.
Purpose of the Study:
- To investigate the role of specific antigens in bullous pemphigoid pathogenesis.
- To explore the function of 230 kD and 180 kD transmembrane proteins in epithelial adhesion.
Main Methods:
- Analysis of antibody responses in affected tissues.
- Identification and characterization of antigens involved in blister formation.
- Review of recent studies defining antigen sequences and cDNA encoding.
Main Results:
- Autoantibodies in BP target unique 230 kD and 180 kD hemidesmosome proteins.
- These antigens are crucial for stratified squamous epithelial cell adhesion.
- Recent structural data provides insights into antigen function.
Conclusions:
- Bullous pemphigoid is considered an autoimmune disease mediated by antihemidesmosome autoantibodies.
- The binding of these autoantibodies to specific antigens is strongly suspected to cause cutaneous lesions.
- Further research is needed to definitively prove the causative role of these autoantibodies in BP pathogenesis.