Study Finds Significant Association Between Bullous Pemphigoid and Neurologic Disorders
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Study Finds Significant Association Between Bullous Pemphigoid and Neurologic Disorders

CHICAGO -- November 15, 2010 -- Individuals with bullous pemphigoid appear more likely to have a diagnosis of neurologic disease, such as dementia and cerebrovascular disease, according to a study published in the November issue of Archives of Dermatology.

“Over the last 2 decades, various neurologic diseases have been described in association with bullous pemphigoid, including cerebrovascular disease, dementia, multiple sclerosis, epilepsy, Parkinson disease, Shy-Drager syndrome and amyotrophic lateral sclerosis,” the authors wrote.

Kathy Taghipour, MD, Oxford Radcliffe Hospitals, Oxford, United Kingdom, and colleagues assessed 90 consecutive patients with bullous pemphigoid and 141 controls without the condition. Medical histories were reviewed for the presence of neurologic disease, defined as a confirmed diagnosis by a hospital physician; positive imaging findings; or considerable functional disability due to mental impairment.

Among patients with bullous pemphigoid, 42 (46%) had at least 1 neurologic disease, compared with 16 controls (11%). Four major neurologic diseases were observed among these patients: cerebrovascular disease, dementia, Parkinson’s disease, and epilepsy. However, only rates of cerebrovascular disease and dementia were significantly greater among patients than among controls.

Of the 36 patients with accurate information about the timing of their diagnoses, bullous pemphigoid was diagnosed after neurologic disease in most (26 of 36 [72%]), with a median time of 5.5 years between diagnoses.

“Neurologic symptoms may often be subtle, and the onset of disease may be insidious, leading to diagnostic delay,” the authors wrote. “It is therefore likely that the interval between neurologic disease and bullous pemphigoid is longer than estimated, and it is tempting to speculate that certain neurologic conditions can predispose to bullous pemphigoid.”

The association could be explained by an immunologic cross-reactivity between the skin and the brain, in which neurologic disorders trigger an immune response that affects the skin, the authors noted.

“The relationship between bullous pemphigoid and neurologic disease has been the subject of numerous case reports, but epidemiological data are scarce, and this subgroup of patients with bullous pemphigoid have not been further characterised,” they concluded. “We have demonstrated that there is a significant association between bullous pemphigoid and neurologic disorders, in particular cerebrovascular disease and dementia, and that neurologic disease may be a predisposing factor for bullous pemphigoid. The mechanism by which neurologic disease may trigger bullous pemphigoid remains obscure and requires further investigation.”

SOURCE: Archives of Dermatology


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