Elsevier

Dermatologic Clinics

Volume 37, Issue 2, April 2019, Pages 215-228
Dermatologic Clinics

Treatment Update of Autoimmune Blistering Diseases

https://doi.org/10.1016/j.det.2018.12.003Get rights and content

Section snippets

Key points

  • Rituximab has become a conventional treatment of pemphigus with an FDA labeled indication in 2018.

  • Anecdotal data indicate that Rituximab is also a promising treatment of autoimmune subepidermal blistering diseases.

  • Rituximab can be used as a rescue treatment in recalcitrant autoimmune blistering diseases in children.

  • Progress continues to be made in refining standardized disease severity scores for autoimmune blistering diseases.

  • Numerous new emerging treatments hold promise with biologics and

Efficacy and safety

The first report of the successful use of rituximab for the treatment of pemphigus vulgaris (PV) was in 2002. Salopek and colleagues7 reported a case of recalcitrant, life-threatening PV with nearly 100% body surface area (BSA) involvement failing to respond to multidrug immunosuppressive therapy.7 The mechanism through which rituximab affects PV is 2-fold: B-cell progenitors of autoantibody-secreting plasma cells are depleted and desmoglein (Dsg)-specific CD4+ T cells are downregulated.8

Since

Next-generation anti-CD20 monoclonal antibodies

New generations of anti-CD20 monoclonal antibodies that are either humanized or fully human have been developed in recent years to circumvent the immunogenicity associated with rituximab. The degree of associated immunogenicity was alleged to embody a decisive role in determining the efficacy and tolerability of the treatment.66 Other rationales motivating the production of these agents are to alleviate the adverse events observed with rituximab exposure and to reduce its costs by using

Summary

There have been significant advances in the treatment of AIBD, and experts see great promise in rituximab with regard to good clinical outcomes and safety profile. In addition, molecules directed against several immunologic pathways have offered possibilities for future implementation in new therapies for these tissue-specific autoimmune diseases.

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    Disclosure: The authors have nothing to disclose.

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