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

Dermatol Ther. 2018 Nov;31(6):e12726. doi: 10.1111/dth.12726. Epub 2018 Oct 3.


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.

Keywords: epidermolysis bullosa acquisita; rituximab.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged, 80 and over
  • Drug Therapy, Combination
  • Epidermolysis Bullosa Acquisita / diagnosis
  • Epidermolysis Bullosa Acquisita / drug therapy*
  • Epidermolysis Bullosa Acquisita / immunology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Remission Induction
  • Rituximab / therapeutic use*
  • Skin / drug effects*
  • Skin / immunology
  • Skin / pathology
  • Treatment Outcome


  • Immunosuppressive Agents
  • Rituximab