title: Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?
abstract: We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.
authors.affiliation: Department of Dermatology, Bichat-Claude-Bernard Hospital, 46, rue Henri-Huchard 75018 Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: Foureur
authors.forename: V
authors.initials: V
authors.lastname: Descamps
authors.forename: B
authors.initials: B
authors.lastname: Lebrun-Vignes
authors.forename: C
authors.initials: C
authors.lastname: Picard-Dahan
authors.forename: M
authors.initials: M
authors.lastname: Grossin
authors.forename: S
authors.initials: S
authors.lastname: Belaich
authors.forename: B
authors.initials: B
authors.lastname: Crickx
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000276:immunology; D001324:Autoantigens / Q000235:genetics / Q000276:immunology; D002352:Carrier Proteins; D016022:Case-Control Studies; D003094:Collagen / Q000235:genetics / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000276:immunology; D000071821:Dystonin; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000276:immunology; D009422:Nervous System Diseases / Q000150:complications* / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D024041:Non-Fibrillar Collagens; D010291:Paresis / Q000150:complications* / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D010391:Pemphigoid, Bullous / Q000209:etiology* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D015995:Prevalence; D011379:Prognosis; D017385:Sequence Homology; D000097805:Collagen Type XVII
chemical_list: 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