Bullous pemphigoid and amyotrophic lateral sclerosis - A new clue for understanding the bullous disease?

Background: Bullous pemphigoid (BP) occurs in many patients with multiple s clerosis. Isolated cases of BP in patients with other neurological disorder s further support a pathogenic association between cutaneous and neurologic al diseases; Any description of BP in patients with amyotrophic lateral scl erosis is lacking. Observations: We studied a French population of 168 patients with typical a myotrophic lateral sclerosis. Among these, 3 had clinical and histological features of HP. The mean age of the patients was 54 years. None was known t o have autoimmune disorders. Results of irnmunoblot analysis disclosed both anti-BP antigen 1 and anti-BP antigen 2 antibodies. Conclusions: Bullous pemphigoid seems to be unexpectedly associated with am yotrophic lateral sclerosis. On the basis of the cases presented herein, we discuss the epidemiological significance of the association and the possib le interrelation between BP antigen 1 and neurofilaments in the pathogenesi s of both disorders.