Neurological Disorders in Patients with Bullous Pemphigoid

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.