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A New Clue for Understanding the Bullous Disease?

Olivier Chosidow, MD, PhD; Valérie Doppler, MD; Gilbert Bensimon, MD, PhD; Pascal Joly, MD, PhD; François Salachas, MD; Lucette Lacomblez, MD; Catherine Prost, MD, PhD; William Camu, MD; Camille Francès, MD; Serge Herson, MD; Vincent Meininger, MD

Arch Dermatol. 2000;136:521-524.

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.

Observations  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.

From the Departments of Internal Medicine (Drs Chosidow, Francès, and Herson), Pharmacology (Drs Doppler, Bensimon, and Lacomblez), and Neurology (Drs Salachas and Meininger), Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, Université Paris VI, and the Department of Dermatology, Hôpital Saint-Louis (Dr Prost), Paris, France; the Department of Dermatology, Hôpital Charles Nicolle, Rouen, France (Dr Joly); and the Department of Neurology, Hôpital Gui de Chauliac, Montpellier, France (Dr Camu).

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