DR Medhat MRCP

Pemphigus vulgaris
- Is an autoimmune disease caused by antibodies directed against desmoglein, a cadherintype
epithelial cell adhesion molecule.
- It is more common in the Ashkenazi Jewish population.
سؤال مهم Blisters/bullae
No mucosal involvement: bullous pemphigoid
Mucosal involvement: pemphigus vulgaris
• Features
Mucosal ulceration is common and often the presenting symptom.
Oral involvement is seen in 50-70% of patients.
Skin blistering :
o Flaccid, easily ruptured vesicles and bullae.
o Lesions are typically painful but not itchy.
o These may develop months after the initial mucosal symptoms.
o Nikolsky's sign describes the spread of bullae & exfoliation of its
superficial layer away from deep layer of skin following application of
horizontal , tangential pressure to the skin.
Acantholysis on biopsy (i.e loss of intercellular connection between keratocytes)
Oral ulceration Mucosal blistering Skin blistering Nikolsky`s sign
• Management:
Steroids.
Immunosuppressants.
Acantholysis & separation of keratocytes
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