Clinical Response of Severe Mechanobullous Epidermolysis Bullosa Acquisita to Combined Treatment With Immunoadsorption and Rituximab (Anti-CD20 Monoclonal Antibodies)

Abstract

Background: Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disorder with mucocutaneous involvement, skin fragility, and tendency to scarring. The mechanobullous form of EBA has a chronic relapsing course and is difficult to treat. We describe herein the therapeutic response of 2 patients with recalcitrant mechanobullous EBA to combined treatment with immunoadsorption and rituximab, an anti-CD20 monoclonal antibody that induces depletion of B cells in vivo. Observations: Two patients with mechanobullous EBA received combined treatment with immunoadsorption and rituximab, resulting in an almost complete clinical remission in one patient and stable disease in the other patient. In the patient with complete remission, prolonged B-cell depletion and clinical improvement with disappearance of mucocutaneous erosions

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

“…14,25,31 Concominant systemic therapy used during rituximab treatment have included prednisone, dexamethasone, mycophenolate mofetil, azathioprine, cyclophosphamide, colchicine, methotrexate, 41 IVIg, plasmaphoresis, 42 daclizumab (anti-CD25 antibody), 34 and immunoabsorption. 13 As noted earlier, in this review we found 69.01% of autoimmune blistering patients treated with rituximab showed a complete response and 25.35% showed partial response. Collectively, 67 (94.37%) of patients showed clinical improvement.…”

“…12 Niedermeier and colleagues also reported a patient with epidermolysis bullosa acquisita with complete remission after rituximab treatment, had clinical improvement which paralleled the decline of anti-basement membrane autoantibody titer. 13 Furthermore, Ahmed and colleagues and Schmidt and colleagues reported reduction of autoantibody titers during the treatment with combined rituximab and IVIg. 14,15 To what extent does the IVIg contribute to the autoantibody titer reduction is not clear and cannot be determined by the published data derived from this combined rituximab/IVIg study.…”

“…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 …”

“…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".…”

Plasmapheresis/immunoadsorption in patients with pemphigus

“…Plazmaferez ve immünadsorpsiyon, Goodpasture sendromu, myastenia gravis ve romatoid artrit gibi antikor aracılı hastalıkların tedavisinde kullanılmaktadır. Plazmaferez pemfigus vulgariste ilk kez 1978 yılında, immünadsorbsiyon ise PNP 1998 yılında kullanılmıştır 8 ; PF, büllöz pemfigoid 9 , pemfigoid gestasyones 10,11 , anti p-200 pemfigoidi 12 ve akiz epidermolizis bülloza 13 tedavisinde de kullanıldığına ilişkin yayınlar mevcuttur. Literatürde PV'de plazmaferez kullanımına dair mevcut olan tek kontrollü çalışma Guillaume ve ark.…”