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Revista de neurologia · Nov 2008
Case Reports[Bullous pemphigoid and amyotrophic lateral sclerosis].
- D A García-Estévez, G Peón-Currás, and F Bal-Nieves.
- Unidad de Neurología, Hospital Comarcal de Monforte de Lemos, 27400 Monforte de Lemos, España. daniel.apolinar.garcia.estevez@sergas.es
- Rev Neurol. 2008 Nov 16; 47 (10): 525-6.
IntroductionBullous pemphigoid is the most frequent blistering disease and has been found associated to several neurological diseases, including amyotrophic lateral sclerosis.Case ReportA 63-year-old male with bulbar-onset amyotrophic lateral sclerosis who presented clinical and histological signs and symptoms of bullous pemphigoid.ConclusionsThe 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.
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