Concurrent autoimmune blistering diseases in VEXAS syndrome: a report of two cases

• DOI: 10.1093/ced/llae288
• Publication Date: 2024-07-24T01:39:58Z
• Authors: Aaron Bao; Ananya Munjal; Pooja S Reddy; Eugene Park; Daniel R Synkowski; Jaroslaw J Jedrych; Jun Kang
• Keywords: Dermatitis herpetiformis; Immune Dysregulation; Pemphigus vulgaris

VEXAS (vacuoles, E1-enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently characterized autoinflammatory condition driven by myeloid cell dysregulation. We report two patients with concurrent diagnoses of and autoimmune blistering diseases – pemphigus vulgaris dermatitis herpetiformis highlighting previously undescribed association between innate immune dysregulation autoantibody-mediated skin diseases.