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[The mosaic of pemphigus]
1Autoimmune Disease Research Unit, Department of Internal Medicine B, Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University.
Harefuah
|January 5, 2002
Summary
Pemphigus is an autoimmune blistering disease targeting desmogleins. Advancements reveal a two-step antibody development process and Major Histocompatibility Complex (MHC) links to disease susceptibility.
Area of Science:
- Immunodermatology
- Autoimmune diseases
Context:
- Pemphigus is a severe autoimmune blistering disease affecting skin and mucous membranes.
- Pemphigus vulgaris and pemphigus foliaceus are the classic forms, differing in lesion localization.
Purpose:
- To review recent advancements in the etiology and pathogenesis of pemphigus.
- To elucidate the role of desmogleins and Major Histocompatibility Complex (MHC) in pemphigus development.
Summary:
- Desmogleins, crucial for cell adhesion, are identified as autoantibody targets in pemphigus.
- Disease localization differences correlate with desmoglein distribution and expression.
- Pemphigus pathogenesis involves a two-step autoantibody increase, with MHC-linked susceptibility explained by selective self-peptide presentation.
Impact:
- Understanding desmoglein targets and MHC associations provides insights into pemphigus pathogenesis.
- Identifying disease-specific peptides may aid in discovering microbial triggers for pemphigus.