Fulminante, rituximab-resistente, mucocutane pemphigus vulgaris

Pemphigus vulgaris is an autoimmune disease mediated by auto-antibodies against desmoglein 1 and 3. First line treatment for pemphigus consists of systemic corticosteroids and anti-CD20 therapy (rituximab) to eliminate B-cells. Since 2005, more than 100 patients with pemphigus have been treated with rituximab at the Center for Blistering Diseases of the University Medical Center Groningen. Until recently, all patients responded to rituximab therapy. In this report we present a case of fulminant mucocutanous pemphigus vulgaris that did not respond to rituximab. We did not detect antibodies against rituximab in patient's serum. After the second, life threatening flair it was decided to eliminate plasma cells (CD20 negative) with a standard multiple myeloma protocol. This protocol consists of bortezomib (Velcade), cyclophosphamide and dexamethasone regimens followed by autologous stem cell transplantation. After more than 227 days spent in the ward and five VCD regimens, the patient went into disease remission. Currently the patient is waiting for an autologous stem cell transplantation to increase the likelihood of achieving a durable complete remission