An immunogenetic study of bullous pemphigoid with mucosal involvement in two siblings

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease mostly affecting the elderly. Patients with BP usually have autoantibodies against BP180 and/or BP230, which are major components of hemidesmosomes. Although the etiology is unclear, many studies have shown that onset of BP is associated with human leukocyte antigen (HLA) [1]. Here, we report two sibling BP patients carrying the HLA-DQB1*03:01 and HLA-DRB1*04 alleles.Case 1: A 95-year-old Japanese man presented with a 1-month [...]