The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, subepidermal blistering disease affecting the skin and mucous membranes that often remains refractory to standard immunosuppressive therapy. We present three original cases and a review of the literature of 20 cases of refractory EBA treated with rituximab as monotherapy or in combination with other agents. Complete control (with or without therapy) and remission were seen in 56% of patients treated with rituximab monotherapy and 75% of patients treated with rituximab and immunoadsorption (IA). We conclude EBA refractory to standard immunosuppressive therapy may show a more favorable long‐term response to the addition of rituximab; and rituximab in combination with intravenous immunoglobulin or IA may provide utility in terminating acute disease. Additional data are needed to evaluate the safety and long‐term outcomes of rituximab‐based treatment.

Epidermolysis bullosa acquisita treated with rituximab

“…EBA is a rare acquired, mucocutaneous, subepidermal, autoimmune blistering disease, which results in vesicle and bullae formation on the skin and erosions on the mucous membranes. [1][2][3][4][5] EBA can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. [1][2][3] It usually appears in adulthood (with a median age of 50 years), with no known predilection to sex.…”

“…[1][2][3] Once the diagnosis is established, however, it is challenging to find optimal treatment for the patient. [1][2][3][4][5] Multiple drugs have been used in the treatment of EBA, resulting in varying degrees of response; the most frequently reported agents include systemic corticosteroids, antiinflammatory and immunosuppressive agents. [1][2][3][4][5] Figure 1 Vesiculobullous lesions involving the oral mucosa.…”

“…Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, azathioprine, photopheresis, plasmapheresis, infliximab, rituximab and intravenous immunoglobulins. [1][2][3][4][5] EBA, especially in its classic form, is characterised by poor response to immunosuppressive therapies. [1][2][3][4][5] There have been developments in newer treatment modalities that have achieved some success.…”

Efficacy of intravenous immunoglobulins for laryngopharyngeal lesions and upper airway obstruction in epidermolysis bullosa acquisita

“…Epidermolysis bullosa acquisita patients, especially with IgA deposits on basement membrane in direct immuno‐fluorescence, often exhibit mucosal lesions . For the laryngeal lesions of EBA, extensive systemic treatment, such as pulse methylprednisolone therapy and immunosuppressive agents, is required to prevent the serious complications such as airway stenosis . However, mucosal lesions of EBA are often refractory to these therapies.…”

“…However, mucosal lesions of EBA are often refractory to these therapies. Rituximab, an anti‐CD20 antibody that depletes B cells, is suggested to be a novel therapeutic option for intractable EBA, but its increased risk of infection has been concerned …”