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Updated: Jun 28, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Bullous pemphigoid and amyotrophic lateral sclerosis]
D A García-Estévez1, G Peón-Currás, F Bal-Nieves
1Unidad de Neurología, Hospital Comarcal de Monforte de Lemos, 27400 Monforte de Lemos, España. daniel.apolinar.garcia.estevez@sergas.es
Introduction:
Bullous pemphigoid is the most frequent blistering disease and has been found associated to several neurological diseases, including amyotrophic lateral sclerosis.
Case Report:
A 63-year-old male with bulbar-onset amyotrophic lateral sclerosis who presented clinical and histological signs and symptoms of bullous pemphigoid.
Conclusions:
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.
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