Dystonin, previously identified as BPAG1 and BP230 in studies of the autoimmune disease bullous pemphigoid [15, 16], was later determined to be a large gene with complex transcripts in both mouse and human, including highly nerve, muscle and skin (epidermal) specific isoforms initially dubbed BPAG1a, BPAG1b and BPAG1e respectively and later named Dst-a, Dst-b and Dst-e [17–20]. This evidence concerns the gene DST and bullous pemphigoid.