Autoimmune encephalitis associated with autoimmune blistering diseases: A case series and retrospective review

• Authors: Andrew R Tadros, Emory University; George Atteh, Emory University; Diana L Vargas, Emory University; Emily Cole, Emory University; Ron Feldman, Emory University
• Journal Title: JAAD Case Reports
• Volume: Volume 37
• Publisher: Elsevier, Inc. | 2023-07-01, Pages 92-97
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1016/j.jdcr.2023.04.030
• Subjects: Health Sciences, Medicine and Surgery
• Keywords: autoimmune blistering skin disease; autoimmune encephalitis; case series; dermatology; retrospective review

Abstract

Autoimmune blistering skin diseases (AIBDs) comprise a group of immune-mediated disorders characterized by the presence of autoantibodies directed against cutaneous epitopes resulting in frequent blister formation. Associations between the most common AIBDs, pemphigus, and pemphigoid and other inflammatory or autoimmune diseases have been described, so-called autoimmune diathesis.1 This diathesis has been well described with regard to pemphigoid and concomitant neurologic inflammatory or autoimmune diseases such as Alzheimer’ disease, Parkinson’ disease, and multiple sclerosis.2,3 These neurocutaneous associations are hypothesized to be, at least in part, caused by autoantibodies directed at both cutaneous and neurologic tissues. Due to their shared embryologic origin, several receptor binding domains are co-expressed across both dermatologic and neurologic tissues (eg, BP180, BP230). Cross-reactive antibodies are believed to play a role in mediating the clinical sequelae related to the skin; although the role in various neurological diseases is still unclear, with circulating anti-BP180, 230 autoantibodies reported in patients with Alzheimer disease and Parkinson disease.4,5