What’s New In Blistering Diseases


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In this issue, I am pleased to serve as an invited editor for this special collection of reviews, titled “What’s New in Blistering Diseases”. There has been tremendous progress in elucidating the pathomechanisms of such diseases, and these research advances promise to lead to therapeutic trials.

For epidermolysis bullosa (EB), a non-lethal mouse model provides us with the opportunity to try various innovative treatments, including cell therapy, protein therapy and gene therapy. It was a wonderful surprise for us to see bone marrow transplantation therapy for dystrophic EB recently conducted in the USA, and we look forward to more details on the treatment mechanism, efficacy and risk of allogenic bone marrow transplantation.

For bullous pemphigoid (BP), a novel mouse model using a collagen-17-humanized mouse is allowing us to evaluate the pathogenicity of human BP autoantibodies and investigate the efficacy of treatment regimens. Elucidation of the target molecule of anti-p200 pemphigoid as laminin gamma-1 is another momentous finding that is covered here.

For pemphigus, a Japan-wide prospective study of i.v. immunoglobulin (IVIG) therapy has clearly shown the efficacy of IVIG when combined with systemic corticosteroid. Although IVIG is not a cheap option, we are pleased that the Japanese government has recognized it for coverage by health insurance. And finally, the fundamental immune dysregulation mechanism that leads to the production of pemphigus autoantibodies is introduced. I hope the reader enjoys these review papers.