Bullous pemphigoid and amyotrophic lateral sclerosis: a new clue for understanding the bullous disease?
BACKGROUND
Bullous pemphigoid (BP) occurs in many patients with multiple sclerosis. Isolated cases of BP in patients with other neurological disorders further support a pathogenic association between cutaneous and neurological diseases. Any description of BP in patients with amyotrophic lateral sclerosis is lacking.


METHODS
We studied a French population of 168 patients with typical amyotrophic lateral sclerosis. Among these, 3 had clinical and histological features of BP. The mean age of the patients was 54 years. None was known to have autoimmune disorders. Results of immunoblot analysis disclosed both anti-BP antigen 1 and anti-BP antigen 2 antibodies.


CONCLUSIONS
Bullous pemphigoid seems to be unexpectedly associated with amyotrophic lateral sclerosis. On the basis of the cases presented herein, we discuss the epidemiological significance of the association and the possible interrelation between BP antigen 1 and neurofilaments in the pathogenesis of both disorders.
Chosidow
O
O
Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, Université Paris VI, France. olivier.chosidow@psl.ap-hop-paris.fr
Doppler
V
V
Bensimon
G
G
Joly
P
P
Salachas
F
F
Lacomblez
L
L
Prost
C
C
Camu
W
W
Francès
C
C
Herson
S
S
Meininger
V
V
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D002352:Carrier Proteins; D003094:Collagen / Q000276:immunology; D003598:Cytoskeletal Proteins; D004359:Drug Therapy, Combination; D000071821:Dystonin; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D015151:Immunoblotting; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D012008:Recurrence; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human