Penfigoide ampolloso y esclerosis lateral amiotrófica

Bullous pemphigoid is the most frequent blistering disease and has been found associated to several neurological diseases, including amyotrophic lateral sclerosis.A 63-year-old male with bulbar-onset amyotrophic lateral sclerosis who presented clinical and histological signs and symptoms of bullous pemphigoid.The association does not seem to occur by chance and we suggest an autoimmune pathogenetic mechanism consisting in a crossed reaction between bullous pemphigoid antigen 1 and the protein dystonin, which is involved in the organisation/integrity of the neuronal cytoskeleton.