Neurological Disorders in Patients with Bullous Pemphigoid

Abstract

Background: Unexpected cases of bullous pemphigoid (BP) have been reported in adult patients with various neurological disorders suggesting a possible relationship between these diseases. Objectives: (1) To determine the prevalence and types of neurological disorders in patients with BP, (2) to assess patients’ functional impairment, and (3) to compare the clinical and biological findings as well as prognosis of BP patients presenting with or without neurological disorders. Methods: BP patients with neurological disorders were selected in a series of 341 consecutive BP patients treated in 20 French Dermatology Departments. Functional impairment was prospectively assessed using the Karnofsky score which is a measure of patients’ general condition. Results: At least one neurological disorder was present in 123 of the 341 BP patients (36%). They primarily consisted of dementia (n = 68; 20%; 95% CI: 16–25%), cerebral stroke (n = 52; 15%; 95% CI: 4–19%), and/or Parkinson’s disease or parkinsonism (n = 32; 9%; 95% CI: 7–13%). BP patients with neurological disease were older than patients without neurological disease (83.8 ± 7.5 years vs. 79.3 ± 10.3 years, p < 10^–4). They also had a lower Karnofsky score (47 ± 19% vs. 74 ± 20%, p < 10^–4). One-year overall survival rates of the two groups were 50.8% (95% CI: 41.8–59.7) and 78.7% (95% CI: 73.0–84.2), respectively (p < 10^–4). In contrast, the number of bullae and main biological features at baseline were not different between the two groups of patients. Conclusion: This study demonstrated a high frequency of neurological disorders, particularly dementia, in BP patients. Most of these patients had a severe functional impairment and a poor prognosis.

Otoimmün büllü hastalıkların klinik ve demografik özellikleri: 85 hastanın retrospektif analizi

“…Yine daha önceden rapor edilmiş iki pemfiguslu olgumuzdan birisinde hastalık öncesi çok sayıda (100'e yakın) arı sokması öyküsü mevcutken diğerinde ise takipleri sırasında mide adenokarsinomu gelişti 24,25 . Büllöz pemfigoid otoimmün, nörolojik ve maliniteler gibi çeşitli hastalıklar ile birlikte görülebilir 5,20,26 . BP'nin malinitelerle olan birlikteliği halen tartışmalı olsa da, bu birlikteliğe ait yayınlar az değildir [27][28][29] .…”

“…Çalışmamızda BP'li üç olguya (%13,64) malinite eşlik ediyordu: Bir olguda kemik metastazlı meme kanseri 29 , diğer bir olgumuzda renal karsinoma ve bir olguda da lenfoma saptandı. BP'ye eşlik edebilecek diğer bir hastalık nörolojik hastalıklardır 20,26 . Literatürde çeşitli nörolojik hastalığı olan olgularda BP gelişimi bildirilmiş ve bu 2 hastalık grubu arasında bir ilişki olabileceği öne sürülerek, bu olgularda BP prognozunun daha kötü olduğu vurgulanmıştır 20 .…”

Síndrome Demencial como Fator de Risco para Penfigoide Bolhoso

“…5,7 A relação causal entre as doenças do foro neurológico e a ocorrência de penfigoide bolhoso tem sido muito investigada nos últimos anos. [1][2][3][4][5]7 Embora a sua patogénese ainda não esteja totalmente esclarecida, acredita-se que os processos neurodegenerativos possam predispor o desenvolvimento de PB, através da ativação de mecanismos autoimunes, que produzem autoanticorpos contra antigénios presentes nas células cutâneas e do sistema nervoso central. [1][2][3][4] Estas observações são reforçadas pelo facto de ambos os tecidos (cutâneo e sistema nervoso) serem originados a partir da crista neural.…”

“…1,2 Vários estudos recentes demonstraram a associação entre a ocorrência de PB em doentes com síndromes neurológicas como esclerose múltipla, doença de Parkinson, epilepsia e doenças cerebrovasculares. [1][2][3][4][5] Os mecanismos fisiopatológicos subjacentes a este processo ainda não estão totalmente esclarecidos. A hipótese mais plausível tem como base a reatividade imunológica cruzada entre autoanticorpos das células cutâneas e do sistema nervoso central, potenciada pela perda de tolerância imunológica que está associada ao processo de envelhecimento e ao aparecimento de sín-dromes demenciais.…”

Cytotoxic and antigen presenting cells, and non-basement membrane zone pathology in a case of bullous pemphigoid

“…The study sample included 390 patients with BP, versus 1950 matched subjects in a comparison group [14]. Other authors have also reported statistically significant cardiovascular and neurologic alterations between patients affected by BP and matched control groups [14][15][16][17][18]. As we have previously noted, most classic skin immunofluorescence studies have been performed with monofluorochome techniques, frequently utilizing FITC.…”