Table I.

Therapeutic options for the treatment of pemphigus vulgaris.

Therapy	Method
First-line therapy	Corticosteroids (Prednisolone) Start with 1 mg/kg per day in severe cases 0.5–1 mg/kg per day in milder cases Doses may be increased by 50–100% every 5–7 days if blistering continues After installation of the remission period, the doses are gradually decreased (5–10 mg prednisolone/2 weeks down to 20 mg daily, then by 2–5 mg every 2–4 weeks down to 10 mg daily) Add an adjuvant immunosuppressant: Azathioprine 2–3 mg kg - 1 per day Mycophenolate mofetil 2–3 g per day Rituximab (rheumatoid arthritis protocol, 291 g infusions, 2 weeks apart)
Second-line therapy	If first-line treatment does not work, switch to alternate corticosteroid-sparing agent (azathioprine, mycophenolate mofetil or rituximab)
Third-line therapy	Cyclophosphamide Immunoadsorption Intravenous immunoglobulin Methotrexate Plasmapheresis or plasma exchange

Adapted from British Association of Dermatologists guidelines for the management of pemphigus vulgaris 2017 (26).