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ALL / Dermatology

Pemphigus vulgaris

Pemphigus vulgaris

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.

Features
  • 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
  • acantholysis on biopsy

Image used on license from DermNet NZ
Pemphigus vulgaris. Flaccid bullae and oral erosions with secondary candidiasis
Image used on license from DermNet NZ
Image used on license from DermNet NZ

Management
  • steroids are first-line
  • immunosuppressants
+PassMedicine Notes

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.

Features
  • 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
  • acantholysis on biopsy
Management
  • steroids are first-line
  • immunosuppressants


Media
Pemphigus vulgaris
Osmosis - YouTube
👍 22 👎 4
Pemphigus vulgaris
YouTube
👍 22 👎 4
Links
British Association of Dermatologists
👍 7👎 3
2017 Guidelines for the management of pemphigus vulgaris
DermNet NZ
👍 5👎 3
Pemphigus vulgaris
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