Clinical and Immunologic Factors Associated With Bullous Pemphigoid Relapse During the First Year of Treatment

Fichel, F.; Barbe, Coralie; Joly, P.; Bédane, C.; Vabres, P.; Truchetet, F.; Aubin, F.; Michel, Catherine; Jegou, J.; Grange, Florent; Antonicelli, Frank; Bernard, Philippe

doi:10.1001/jamadermatol.2013.5757

Cited by 73 publications

(98 citation statements)

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“…In a previous study, anti-BP180 and anti-BP230 autoantibodies were detected by ELISA at baseline, and then at months 3 and 6, and correlated both with BPDAI score and Autoimmune Bullous Skin Disorder Intensity Score [16]. At baseline, BPDAI mean scores were similar in both studies, and anti-BP180 and anti-BP230 ELISA values were consistent with previous reports [10,22,27,28,29]. Likewise, at baseline, we did not find a correlation between BPDAI and anti-BP230 ELISA values, which is consistent with the results obtained by Patsatsi et al [16].…”

Section: Discussionsupporting

confidence: 87%

“…This is likely due to the use of superpotent topical steroids for the treatment of the majority of our patients, instead of systemic prednisolone in the series of Patsatsi et al [16]. However, superpotent topical corticosteroids alone were clinically effective, as illustrated by the control of the disease of all BP patients at day 30, in accordance with previous studies [18,19,29]. …”

Section: Discussionsupporting

confidence: 87%

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Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

et al. 2014

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Background: Recently, a consensus Bullous Pemphigoid Disease Area Index (BPDAI) was proposed to measure therapeutic outcomes in bullous pemphigoid (BP). Objective: To compare BPDAI with other clinical parameters of disease activity at baseline and to describe the variations of BPDAI during the initial phase of treatment. Methods: Thirty BP patients were included and followed for 1 year. BPDAI was assessed at baseline and on days 30, 90 and 360 by the same investigator. Concomitantly, the number of daily new blisters, the skin surface area of erythematous/eczematous/urticarial plaques and blisters/erosions, total lesion area (TLA), pruritus score and mucosal involvement were recorded. Results: At baseline, BPDAI was 46.7 ± 25 (mean ± SD); it was well correlated with erythematous/eczematous/urticarial skin surface (r = 0.63), TLA (r = 0.83), number of daily new blisters (r = 0.7; p ≤ 0.0002) and anti-BP180 autoantibodies (r = 0.49; p = 0.006), but not with anti-BP230 autoantibodies. For the 8 patients with severe BP at baseline, the mean BPDAI was 76.5, versus 35.9 for moderate BP (p = 0.0007). A value of 56 was proposed as a cut-off value for severe BP. BPDAI decreased to 11.9 ± 8.7, 10.7 ± 12.7 and 2.5 ± 4.1 on days 30, 90 and 360, respectively. Conclusion: BPDAI rapidly decreased during the early treatment stage of BP with variations almost totally conditioned by the skin activity component.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionsupporting

confidence: 87%

Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

et al. 2014

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“…Anti-BP180 antibodies detected by immunoblotting were found to be associated with death in the first year [16]. The role of ELISA-BP180 test for predicting relapse during the first year of treatment [17] or after treatment cessation was previously described, and a threshold of 27 UA/ml was found to be associated with relapse at 12 months after treatment cessation. Yet, our current data did not confirm the association of ELISA-BP180 and relapse.…”

Section: Discussionmentioning

confidence: 99%

“…The test is sensitive and specific (>90%) at the time of diagnosis [13,14] and results parallel the clinical activity of BP during treatment [15,16,17,18]. Values ≥27 arbitrary units (AU)/ml (3 times the 9 AU/ml threshold of the MESACUP BP180 and BP230 kit [MBL, Nagoya, Japan]) represent a major prognostic factor for predicting relapse after treatment cessation similar to persistent positive direct immunofluorescence (DIF) [6].…”

Section: Introductionmentioning

confidence: 99%

“…Several studies involving the detection of serum anti-BP230 autoantibodies with ELISA (ELISA-BP230) at diagnosis showed a positive correlation with IIF titers and lower sensitivity than ELISA-BP180 (about 60%), but high specificity (>90%) [14,18,19]. In contrast with ELISA-BP180, ELISA-BP230 values do not correctly correlate with disease activity at diagnosis [15], and their usefulness for immunological follow-up of the disease is poorer than that of ELISA-BP180 [15,17,18]. …”

Section: Introductionmentioning

confidence: 99%

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Positive Direct Immunofluorescence Is of Better Value than ELISA-BP180 and ELISA-BP230 Values for the Prediction of Relapse after Treatment Cessation in Bullous Pemphigoid: A Retrospective Study of 97 Patients

Ingen‐Housz‐Oro

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Belmondo³

et al. 2015

Dermatology

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Background: ELISA-BP180 values and direct immunofluorescence (DIF) are prognostic factors for relapse after treatment cessation in bullous pemphigoid (BP). Objective: To determine the relevance of ELISA-BP230 antibodies for predicting relapse 6 months after treatment cessation. Methods: We retrospectively selected patients with BP and available data from ELISA-BP180 and -BP230 and DIF performed at treatment cessation. The rate of relapse was calculated at 6 months. We compared ELISA-BP180 and -BP230 values and DIF in patients with relapse and remission. Results: We included 97 patients. At 6 months, 25.6% of patients showed relapse. The proportion of patients with an ELISA-BP230 value ≥27 UA/ml was higher, but not significantly, for those with relapse than for those with remission (p = 0.11). The frequency of positive DIF findings was significantly higher for patients with relapse (p = 0.005). Conclusion: DIF is of better value than ELISA-BP180 and -230 tests to predict relapse after treatment cessation in BP.

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Association of Transient Palmoplantar Keratoderma With Clinical and Immunologic Characteristics of Bullous Pemphigoid

et al. 2019

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IMPORTANCE Development of transient palmoplantar keratoderma (PPK) with bullous pemphigoid (BP) has only been described in 2 isolated case reports. The clinical significance and the pathophysiologic mechanisms of this association are unknown. OBJECTIVE To examine the clinical characteristics and immunological profile of patients with BP who develop transient PPK and analyze therapeutic options and outcomes.DESIGN, SETTING, AND PARTICIPANTS In this case series, patients with BP who developed acquired, transient PPK, and were treated at a single institution from January 1, 2015, through December 31, 2017, were studied. MAIN OUTCOMES AND MEASURES Clinical and immunological activity of BP, treatment administrated before and after PPK appearance, and patient outcomes.RESULTS Six patients with BP and transient PPK were identified and included in the study. There were 5 women and 1 man with a mean age of 72 years. At baseline, all patients had a generalized, multibullous BP and high serum anti-BP180 antibodies (mean, 130 U/mL; range, 73-150), whereas anti-BP230 antibodies were elevated in only 1 case. The PPK appeared a mean 6.2 (range, 2-12) months after BP diagnosis, following a prolonged period of disease activity with recurrent flares. When the PPK occurred, BP was uncontrolled on therapy (mean Bullous Pemphigoid Disease Activity Index [BPDAI] score, 57; range, 34-105; mean anti-BP180 antibodies titer, 122 U/mL; range, 81-150). On administration of additional systemic immunosuppressive therapies, the PPK healed progressively in a mean 4.3 months (range, 2-9), along with BP clinical remission in 4 of 6 patients. No relationship was found between PPK occurrence and anti-BP180/230 antibodies profiles. In contrast, blister fluids collected at the time of PPK displayed a much higher level of interleukin 1β (IL-1β) compared with those collected in the absence of PKK. Expression of IL-17A, IL-17F, and IL-22 was also enhanced in the blister fluid of patients with BP who had PPK CONCLUSIONS AND RELEVANCE To our knowledge, this is the first report of 6 cases of BP with transient PPK with extensive immunological investigation. The PPK appeared after a prolonged period of clinical BP activity punctuated with recurrent relapses, was transient, and healed after BP control with additional immunosuppressive therapy. Enhanced expression of a particular cytokine panel in the blister fluid at time of PPK could support keratinocyte proliferation as described in patients with psoriasis. Transient PPK could represent a clinical marker of severe, treatment-resistant BP.

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Clinical and Immunologic Factors Associated With Bullous Pemphigoid Relapse During the First Year of Treatment

Cited by 73 publications

References 50 publications

Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

Positive Direct Immunofluorescence Is of Better Value than ELISA-BP180 and ELISA-BP230 Values for the Prediction of Relapse after Treatment Cessation in Bullous Pemphigoid: A Retrospective Study of 97 Patients

Association of Transient Palmoplantar Keratoderma With Clinical and Immunologic Characteristics of Bullous Pemphigoid

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