A possible association between BP230-type bullous pemphigoid and dementia: a report of two cases in elderly patients

Zheng, Miao; Ujiie, Hideyuki; Muramatsu, Ken; Sato‐Matsumura, Kazuko C.; Maeda, Takuya; Ujiie, Inkin; Iwata, Hiroaki; Izumi, Kiyotaka; Nishie, Wataru; Shimizu, Hiroshi

doi:10.1111/bjd.16249

Cited by 7 publications

(3 citation statements)

References 9 publications

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“…Although the pathogenicity of anti-BP230 Abs remains undetermined, a recent study suggested the potential pathogenic role of anti-BP230 Abs [8]. Two cases of elderly BP patients with dementia in which the autoAbs reacted to BP230 but not to BP180 were reported [9]. In contrast, anti-BP230 Abs are rarely detected in DPP4i-BP patients.…”

Section: Discussionmentioning

confidence: 99%

A case of anti-BP230 antibody-positive bullous pemphigoid receiving DPP-4 inhibitor

Sawada

Kobayashi

et al. 2020

Immunological Medicine

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Bullous pemphigoid (BP) is a cutaneous autoimmune blistering disorder. Recently, it has been reported that dipeptidyl peptidase-4 inhibitors ( DPP4i) is associated with the development of BP (DPP4i-BP). Patients with DPP4i-BP have autoantibodies (autoAbs) preferentially targeting full-length BP180, but not the BP180NC16a domain. In this report, we described a case of anti-BP230 antibody (Ab)-positive BP receiving DPP4i. A 78-year-old male with a medical history of type 2 diabetes receiving vildagliptin at 100 mg daily for 38 months was referred to our hospital with itching blisters on his body and extremities. Skin biopsy showed subepidermal bulla with eosinophil infiltration. Direct immunofluorescence staining revealed a linear staining pattern with complement C3 and IgG at the subepidermal basement membrane zone. By laboratory testing, autoAbs against BP180NC16a and full-length BP180 were negative by enzyme-linked immunosorbent assay (ELISA); however, anti-BP230 Abs were positive by ELISA (index: 123.91). His HLA genotype was DQB1 Ã 04:01 and 05:01. Based on these results, we diagnosed the patient with anti-BP230 Abs-positive BP associated with DPP4i. To the best of our knowledge, this is the first case of DPP4i-BP with only anti-BP230 Abs. Further accumulation of DPP4i-BP cases is needed to clarify the relationship between overall features of DPP4i-BP and anti-BP230 Abs.

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Section: Discussionmentioning

confidence: 99%

A case of anti-BP230 antibody-positive bullous pemphigoid receiving DPP-4 inhibitor

Sawada

Kobayashi

et al. 2020

Immunological Medicine

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“…Interestingly, one recent study reported two patients with dementia (AD and dementia with Lewy bodies) that developed mild clinical BP in ages of 91 and 93 years old. These patients had autoantibodies against BP230, but not against BP180, suggesting that some milder inflammatory phenotypes of BP are associated only with BP230 autoantibodies, and that these autoantibodies could be associated with cross-reactive immune response against neural and cutaneous isoforms of dystonin [37].…”

Section: Discussionmentioning

confidence: 91%

The Association Between Frontotemporal Lobar Degeneration and Bullous Pemphigoid

Katisko

Kokkonen

Krüger

et al. 2018

JAD

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Recent studies have shown an epidemiological and immunological association between bullous pemphigoid (BP) and several neurological or psychiatric diseases. Here, our aim was for the first time to specify whether an association exists between BP and frontotemporal lobar degeneration (FTLD). Medical histories of FTLD patients (N=196) were screened for clinical comorbidity and BP180 and BP230 autoantibodies were analyzed in the sera of FTLD patients (N=70, including 24 C9orf72 repeat expansion carriers) by BP180-NC16A-ELISA and BP230-ELISA. One FTLD patient (C9orf72 repeat expansion carrier) had a comorbid diagnosis of BP. Increased levels of serum BP180 autoantibodies (cutoff value >9U/ml) were detected more often in FTLD patients (10.0%) than in controls (4.9%). Moreover, elevated levels of both BP180 and BP230 autoantibodies were found more often in C9orf72 repeat expansion-carrying FTLD than non-carrying patients or controls. However, none of these differences reached a statistical significance likely due to our limited cohort size. In conclusion, our findings suggest that subset of FTLD patients especially with the C9orf72 repeat expansion may have an immunological association with BP.

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“…Next, we were interested in the relation of anti-BP180 and anti-BP230 reactivities with the medication most commonly used by BP patients; that is, DPP4 inhibitors, insulin, antipsychotics, inhibitors of platelet aggregation and L-thyroxine. Following anecdotal evidence for the association of BP with autoantibody response restricted to BP230 and dementia, 62 St€ ander et al reported anti-BP230 seropositivity to be linked to neuropsychiatric comorbidities. In the latter monocentric study with 273 patients that also included patients from the present investigation, only 83 (30%) patients with anti-BP230 IgG levels were evaluated.…”

Section: Discussionmentioning

confidence: 99%

Serum autoantibody reactivity in bullous pemphigoid is associated with neuropsychiatric disorders and the use of antidiabetics and antipsychotics: a large, prospective cohort study

Dikmen

Yilmaz

Benoit

et al. 2022

Acad Dermatol Venereol

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Background Bullous pemphigoid (BP), the by far most frequent autoimmune blistering skin disease (AIBD), is immunopathologically characterized by autoantibodies against the two hemidesmosomal proteins BP180 (collagen type XVII) and BP230 (BPAG1 or dystonin). Several comorbidities and potentially disease‐inducing medication have been described in BP, yet a systematic analysis of these clinically relevant findings and autoantibody reactivities has not been performed. Objective To determine associations of autoantibody reactivities with comorbidities and concomitant medication. Methods In this prospective multicenter study, 499 patients diagnosed with BP in 16 European referral centers were included. The relation between anti‐BP180 NC16A and anti‐BP230 IgG ELISA values at the time of diagnosis as well as comorbidities and concomitant medication collected by a standardized form were analysed. Results An association between higher serum anti‐BP180 reactivity and neuropsychiatric but not atopic and metabolic disorders was observed as well as with the use of insulin or antipsychotics but not with dipeptidyl peptidase‐4 (DPP4) inhibitors, inhibitors of platelet aggregation and L‐thyroxine. The use of DPP4 inhibitors was associated with less anti‐BP180 and anti‐BP230 reactivity compared with BP patients without these drugs. This finding was even more pronounced when compared with diabetic BP patients without DPP4 inhibitors. Associations between anti‐BP180 and anti‐BP230 reactivities were also found in patients using insulin and antipsychotics, respectively, compared with patients without this medication, but not for the use of inhibitors of platelet aggregation, and L‐thyroxine. Conclusion Taken together, these data imply a relation between autoantibody reactivities at the time of diagnosis and both neuropsychiatric comorbidities as well as distinct concomitant medication suggesting a link between the pathological immune mechanisms and clinical conditions that precede the clinically overt AIBD.

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A possible association between BP230-type bullous pemphigoid and dementia: a report of two cases in elderly patients

Cited by 7 publications

References 9 publications

A case of anti-BP230 antibody-positive bullous pemphigoid receiving DPP-4 inhibitor

A case of anti-BP230 antibody-positive bullous pemphigoid receiving DPP-4 inhibitor

The Association Between Frontotemporal Lobar Degeneration and Bullous Pemphigoid

Serum autoantibody reactivity in bullous pemphigoid is associated with neuropsychiatric disorders and the use of antidiabetics and antipsychotics: a large, prospective cohort study

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