Pemphigus Vulgaris Flashcards
What type of disease it it?
Autoimmune
What are the autoantibodies directed against?
Desmosomes - desmoglein 3 (cell adhesion molecule)
Among what population is it more common in?
Ashkenazi Jewish
What is often the presenting symptom?
Mucosal ulceration
How does the skin blistering present?
Flaccid, easily ruptured vesicles and bullae
What symptoms are associated?
Lesions are painful but not itchy
How is it managed?
Prednisolone PO with gradual tapering
Rituximab and IV immunoglobulin in resistant cases
Oral involvement is seen in what percentage of patients?
50-70%
Skin blisters may develop how long after the initial mucosal symptoms?
Months
What are desmosomes?
Protein complexes that bind together cells of the epidermis - most of which found in stratum spinosum
They also bind together mucosal cells
Basal cells are attached to the basement membrane by…
Hemidesmosomes
What type of hypersensitivity reaction is it?
Type 2
What happens when antibodies bind to desmoglein 3/1?
They trigger apoptosis
- cells let go of one another, which leads to bullae formation
Why is this a condition of the the epidermis?
Hemidesmosomes are not affected - the basal layer remains attached to the basement membrane
Are the oral lesions painful?
Yes - can lead to difficulties eating and drinking
