Author: Dr Ian Coulson, Consultant Dermatologist and Editor-in-Chief (2025).
Edited by the DermNet content department.
This 40-year-old man has a one-year history of intermittent oral ulcers, which start as blisters. He has now developed blisters and erosions over the chest.
There is an intact blister on the soft palate.
With the development of skin blisters and erosions that follow oral involvement, one must be highly suspicious of pemphigus vulgaris.
On histological examination, a blister biopsy will show intra-epidermal blistering. At the base of the blister, there are often tombstone-shaped keratinocytes. Acantholysis of epidermal cells will also be seen.
Perilesional direct immunofluorescence will demonstrate intercellular IgG and/or C3 in the epidermis, producing a pattern reminiscent of fishnets. (For more information, see: Pemphigus vulgaris pathology.)
Treatment with systemic corticosteroids, immunosuppressants (eg, azathioprine, mycophenolate mofetil), and rituximab is likely required, as the condition has a high mortality rate if left untreated.
