Oral Jak Inhibitor Upadacitinib Use in Treatment of Pemphigus Foliaceus

Pemphigus foliaceus (PF) is a rare, blistering autoimmune condition that occurs when desmoglein-1 autoantibodies target and lead to loss of intercellular connections, resulting in blister formation on the skin. Current standard of care consists of highly immunosuppressive therapies such as prednisone, rituximab, and mycophenolate mofetil. A 43-year-old male with new-onset PF was treated with upadacitinib, a JAK inhibitor. He saw resolution of his blisters within 12 weeks of treatment and remains in remission from his PF. Our case demonstrates that JAK inhibition may prove to be an effective strategy in preventing dsg-1-triggered blisters. JAK1 inhibitors also may prove to be a safer, less immunosuppressive alternative to the highly immunosuppressive agents available today. Larger studies will be required to study the drug’s efficacy in others with PF.