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Pemphigus: past, present and future
1Department of Dermatology, Hôpital E.-Herriot, Lyon, France.
Dermatology (Basel, Switzerland)
|January 1, 1994
Summary
Pemphigus, a blistering autoimmune disease, involves antibodies targeting desmosomal glycoproteins like desmoglein 3 and desmoglein 1. Associated with tumors and drugs, new forms like IgA pemphigus and paraneoplastic pemphigus expand understanding of dysimmunoreactivity.
Area of Science:
- Immunodermatology
- Autoimmune Bullous Diseases
- Glycoprotein Autoimmunity
Background:
- Pemphigus is a classic autoimmune blistering disease characterized by autoantibodies.
- Key pemphigus antigens are desmosomal glycoproteins: desmoglein 3 (130 kD) in pemphigus vulgaris and desmoglein 1 (160 kD) in pemphigus foliaceus.
- Pemphigus can be triggered by drugs or associated with immune-related tumors like thymoma.
Purpose of the Study:
- To provide an overview of pemphigus as a model autoimmune bullous disease.
- To describe the known pemphigus variants and their associated autoantibodies.
- To introduce newly described forms of pemphigus and their targets.
Main Methods:
- Review of existing literature on pemphigus pathogenesis and clinical variants.
- Characterization of pemphigus autoantigens and their molecular weights.
- Identification of associated conditions and novel pemphigus subtypes.
Main Results:
- Pemphigus autoantibodies target specific desmosomal glycoproteins (desmoglein 1 and 3).
- Pemphigus variants include pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus.
- Autoantibodies can target various desmosomal and cell adhesion molecules, indicating broader dysimmunoreactivity.
Conclusions:
- Pemphigus serves as a key model for understanding autoimmune blistering diseases.
- The spectrum of pemphigus autoantibodies and targets is expanding, including novel forms.
- Understanding pemphigus contributes to the broader concept of dysimmunoreactivity in autoimmune conditions.