[Unexpected positive outcome following rituximab treatment in a patient with pemphigus vegetans resistant to conventional therapies: a case report]. / Evolution spectaculaire d'un pemphigus végétant réfractaire aux thérapies conventionnelles sous Rituximab: à propos d'un cas.

Pemphigus vegetans is a rare form of pemphigus. It accounts for only 2%. There is a broad range of effective options available to treat this disease: topical and/or oral corticosteroids as first-line treatment, immunosuppressants such as Azathioprine, Cyclosporine, Methotrexate, Cyclophosphamide and Mycophenolate mofetil to reduce the side effects of steroids. Some patients are resistant to all these treatments. Rituximab has revolutionized the treatment of pemphigus, pemphigus vegetans in particular. We here report the case of a 42-year old female patient, admitted with pemphigus vegetans confirmed by histology. She initially received corticosteroid therapy associated with immunosuppressive therapy without improvement. Then she was treated with Rituximab and had an unexpected positive outcome.