Characterization of Two Cases of Bullous Pemphigoid Reactive Only with BP230 on Japanese Enzyme-linked Immunosorbent Assays

Subepidermal autoimmune bullous disease affecting predominantly mucocutaneous junctions and the palms with autoantibodies to BP230 and laminin γ1

“…Anti‐BP230 antibodies might be associated with the focal involvement in BP regardless of their pathogenicity . Despite the intracellular localization of BP230, there have been two reported cases of BP reactive only with BP230, although the clinical features were somewhat different from those in our case: one presented with blisters exclusively on the dorsal side of the feet while the other developed blisters over the whole body …”

Four cases of mucous membrane pemphigoid with clinical features of oral lichen planus

“…However, the pathogenic role of the anti‐BP230 antibodies was obscure, because anti‐BP230 antibodies have been considered to be epiphenomenon and non‐pathogenic. On the other hand, our recent study suggested that a relatively large number of BP sera showed only anti‐BP230 antibodies, which may be pathogenic . Therefore, the pathogenic role of anti‐BP230 antibodies should be examined in future studies.…”

Diagnostic accuracy of BP180 NC16a and BP230-C3 ELISA in serum and saliva of patients with bullous pemphigoid

“…We used the ELISA kit introduced by Blöcker et al ., which targets the BP230‐C3 fragment that comprises the C‐terminal portion (amino acids 2326–2649), and our results were comparable with the manufacturer's reported values. We found there was no added diagnostic value of combining the BP230 and BP180 ELISA tests, even though combining BP230 and BP180 ELISA has been shown to slightly increase the sensitivity of BP detection, ranging from 4.2 to 8% in different studies . Recently, it has been shown that 7.8% of BP sera react exclusively to regions of BP180 outside of NC16a, and thus, these would not be identified using the commercially available BP180 ELISA test .…”

“…We found there was no added diagnostic value of combining the BP230 and BP180 ELISA tests, even though combining BP230 and BP180 ELISA has been shown to slightly increase the sensitivity of BP detection, ranging from 4.2 to 8% in different studies. 8,10,20,21 Recently, it has been shown that 7.8% of BP sera react exclusively to regions of BP180 outside of NC16a, and thus, these would not be identified using the commercially available BP180 ELISA test. 22 Our study is limited by the relatively small number of patients; therefore, it is likely that when a larger population of patients with BP is studied, the BP230 ELISA will actually improve the diagnostic sensitivity.…”