Alzheimer’s Disease Identified in a Patient with Bullous Pemphigoid by Dementia Screening Scales
Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder that primarily affects the elderly. It is characterized by the presence of autoantibodies against components of the basement membrane zone (BMZ), particularly BP180 and BP230. While BP is primarily a dermatological condition, recent studies have highlighted its association with various neurological diseases (NDs), including Alzheimer’s disease (AD). This case report describes the identification of AD in a 73-year-old male patient with BP through the use of dementia screening scales, emphasizing the importance of early detection and intervention in such cases.
The patient, a 73-year-old man, presented with erythemas on his trunk and limbs accompanied by significant itching, which had persisted for 11 months. Clinical examination revealed blisters on his hands, and a skin biopsy was performed to confirm the diagnosis. Histological examination showed sub-epidermal blister formation with eosinophilic and lymphocytic infiltration in the dermis. Direct immunofluorescence demonstrated linear deposition of antibodies along the BMZ, while indirect immunofluorescence revealed a high titer (≥1:320) of anti-BMZ antibodies in the patient’s serum. Additionally, the level of anti-BP180 antibody was measured at 102 U/mL. Based on these findings, a definitive diagnosis of BP was established.
During the patient’s hospitalization, clinicians observed signs of cognitive impairment, including bluntness and impaired short-term memory. Further investigation revealed a two-year history of memory decline and a positive family history of dementia, as both his father and sister had been diagnosed with the condition. To assess his cognitive function, the patient underwent screening tests for dementia, including the mini-mental state examination (MMSE) and the Montreal cognitive assessment (MoCA). His MMSE score was 25, and his MoCA score was 19, both indicating impaired cognition. A detailed neuropsychological test battery, administered by a neurologist, confirmed cognitive deficits in multiple domains, including memory, executive function, and visuospatial abilities.
The patient’s apolipoprotein E (ApoE) genotype was determined to be e4/e4, a genetic variant strongly associated with an increased risk of AD. Electroencephalogram (EEG) results were mildly abnormal, and T2-weighted magnetic resonance imaging (MRI) of the brain revealed mild hippocampal atrophy and high-signal intensities in the periventricular white matter. These findings, combined with the clinical presentation and cognitive test results, led to a diagnosis of AD by the neurologist. The patient was prescribed vitamin B6 (10 mg/day), folic acid (5 mg/day), and cobalamin (0.5 mg/day) as part of his treatment regimen.
For the management of BP, the patient was treated with methylprednisolone (48 mg/day) and tripterygium glycosides (60 mg/day). He was advised to follow up regularly with a neurologist for the ongoing management of AD. After 18 months of follow-up, the patient experienced no recurrence of BP, highlighting the effectiveness of the treatment plan.
This case underscores the association between BP and neurological diseases, particularly AD. AD is the most common neurological condition linked to BP, and its gradual progression often results in delayed diagnosis. Dermatologists, who are often the first to encounter patients with BP, play a crucial role in identifying potential neurological abnormalities. Early diagnosis of neurological diseases can lead to more effective healthcare interventions and improved quality of life for patients.
Dementia, especially in its early stages, is often associated with mild symptoms that can be easily overlooked in clinical practice. A comprehensive diagnosis of dementia requires a multidisciplinary approach, including cognitive function tests, neuroimaging, and laboratory evaluations. In this case, the neurologist utilized clinical observations, imaging studies, laboratory tests, and dementia screening scales to diagnose AD. The timely intervention and medication prescribed for the patient may have a positive impact on his prognosis.
The MMSE and MoCA are widely used tools for screening dementia and are employed globally. While the MMSE is effective in distinguishing between normal individuals and those with dementia, it has limitations in differentiating between normal individuals and those with mild cognitive impairment (MCI). The MoCA, on the other hand, has higher sensitivity in detecting MCI, making it a valuable complementary tool. The combined use of these scales can enhance the accuracy of cognitive impairment screening.
This case study highlights the importance of considering the possibility of neurological diseases in patients with BP who exhibit signs of cognitive impairment. Clinicians should be vigilant in assessing the mental health of such patients and utilize appropriate screening tools to facilitate early diagnosis. The use of MMSE and MoCA is recommended for this purpose, as they are sensitive instruments for detecting cognitive deficits.
In conclusion, the identification of AD in a patient with BP through dementia screening scales underscores the interplay between dermatological and neurological conditions. Early detection of neurological abnormalities in patients with BP can lead to timely interventions, potentially improving patient outcomes. Dermatologists and neurologists should collaborate closely to ensure comprehensive care for patients presenting with both dermatological and cognitive symptoms. This case serves as a reminder of the importance of a holistic approach to patient care, particularly in the context of complex, multifactorial diseases.
doi.org/10.1097/CM9.0000000000000285
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