What is Pemphigus vulgaris?

an autoimmune disease which causes blistering and mucosal ulceration

What patient groups is Pemphigus vulgaris most common?

Ashkenazi Jewish population

What are the main clinical features of Pemphigus vulgaris?

• mucosal ulceration is common and often the presenting symptom. Oral involvement is seen in 50-70% of patients
• skin blistering - flaccid, easily ruptured vesicles and bullae. Lesions are typically painful but not itchy. These may develop months after the initial mucosal symptoms.

What are the biopsy findings in Pemphigus vulgaris?

acantholysis

What is the management of Pemphigus vulgaris?

• steroids are first-line
• immunosuppressants

What is the pathophysiology of Pemphigus vulgaris?

antibodies directed against desmoglein 3, a cadherin-type epithelial cell adhesion molecule