Successful Adjuvant Treatment of Recalcitrant Epidermolysis Bullosa Acquisita With Anti-CD20 Antibody Rituximab

Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

“…14 In the previous studies of IVIg treatment for patients with pemphigus, it was reported that the autoantibody titers did reduce during the treatment. 16 Rituximab was originally developed to treat refractory, low-grade, follicular, B cell non-Hodgkins lymphoma 2 , but has been used experimentally in various autoimmune diseases including autoimmune blistering disorders (pemphigus vulgaris, 5-7,9-10,12,14,17-27 pemphigus vegetans, 12 pemphigus foliaceus, 5,8,11,12,28 paraneoplastic pemphigus, [29][30][31][32][33] bullous pemphigoid, 34 and epidermolysis bullosa acquisita 13,35,36 …”

“…34 Epidermolysis bullosa acquisita is another subepidermal blistering disorder with antibodies against collagen VII, a component of the basement membrane zone, and is often recalcitrant to conventional treatment. 35 The autoimmune blistering disorders mentioned above are all conventionally treated with topical corticosteroids along with systemic corticosteroids and/or other systemic immunosuppressants. Most patients respond favorably to conventional regimens; however, a minority of patients fails and other treatment options must be considered.…”

“…32 Thereafter, reports emerged of rituximab used in the treatment of pemphigus vulgaris, 5-7,9,10,12,14,17-27 pemphigus vegetans, 12 pemphigus foliaceus, 5,8,11,12,28 paraneoplastic pemphigus, [29][30][31][32][33] bullous pemphigoid, 34 and epidermolysis bullosa acquisita. 13,35,36 Most commonly, rituximab is administered as a cycle of weekly infusions of 375 mg/m 2 for four weeks. 1 However, other infusion regimens have been described, including repeating a full cycle and/or monthly or every other week "X".…”

Epidermolysis Bullosa Acquisita following Bullous Pemphigoid, Successfully Treated with the Anti-CD20 Monoclonal Antibody Rituximab

“…Recently, Schmidt et al[ 15] reported the case of a patient with recalcitrant EBA that responded to 4 courses of rituximab with a complete remission allowing a progressive discontinuation of colchicine and oral corticosteroids and a reduction of the dose of azathioprine.…”

Rituximab (Anti-CD20 Monoclonal Antibody) – Ultimate or First Choice in Pemphigus?

“…In addition, rituximab has shown promise in single cases of recalcitrant epidermolysis bullosa acquisita [4,5] and mucous membrane pemphigoid [6] .…”