Pemphigus vulgaris is an autoimmune disease caused by antibodies directed against desmoglein 3, a cadherin-type epithelial cell adhesion molecule. It is more common in the Ashkenazi Jewish population.

PC

• 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. Nikolsky's describes the spread of bullae following application of horizontal, tangential pressure to the skin

Investigations

Biopsy:

• Acantholysis - the loss of coherence between keratinocytes of the epidermis that is due to breakdown of desmosomal intercellular attachments and is characteristic of various conditions (as pemphigus and herpesvirus infection) marked by vesicular eruption.

Management

• steroids are first-line
• immunosuppressants