Bullous pemphigoid: Three main clusters defining 3 outcome profiles

Guerrois, Florine; Hassan, Elsa; Bettuzzi, Thomas; Seta, V.; Goulvestre, Claire; Jelti, L.; Belmondo, Thibaut; Wolkenstein, Pierre; Aractingi, S.; Ingen‐Housz‐Oro, Saskia; Dupin, Nicolas

doi:10.1016/j.jaad.2022.04.029

Cited by 7 publications

(9 citation statements)

References 18 publications

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“…21 Multivariate analysis showed that disease control within 4 weeks was related to antibody level rather than treatment regimen. While previous studies have suggested that higher anti-BP180 titers might be related to severe, refractory diseases and adverse outcomes, [30][31][32] anti-BP180 antibodies were mainly IgG4 and IgE, 33 indicating these patients might be more sensitive to IL-4 inhibition therapy. Therefore, dupilumab might be considered as one of the first-line therapies for them.…”

Section: Discussionmentioning

confidence: 82%

Evaluation of Dupilumab in Patients With Bullous Pemphigoid

Zhao,

Wang,

Liang

et al. 2023

JAMA Dermatol

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ImportanceDupilumab is a theoretically novel therapy for bullous pemphigoid (BP). However, its effectiveness and safety have yet to be confirmed in a large-scale study.ObjectiveTo assess the efficacy and safety of dupilumab in patients with BP and evaluate factors that potentially affect short-term and long-term outcomes.Design, Setting, and ParticipantsA retrospective cohort study was conducted from January 1, 2021, to July 31, 2022. The median (IQR) follow-up period was 24.6 (11.5-38.4) weeks. This multicenter study was performed in 6 dermatology departments of the National Autoimmune Bullous Diseases Cooperative Group of China. Adult patients with BP that received 300 mg of dupilumab every 2 weeks following an initial dose of 600 mg were included. Patients were eligible if they had a clinical presentation of BP combined with immunological or pathological evidence. Patients with drug-induced BP, with less than 4 weeks of follow-up, and who received dupilumab or any other biologics within 6 months were excluded.Main Outcomes and MeasuresThe primary outcome was the proportion of patients who achieved disease control within 4 weeks. Disease control was defined as the absence of new lesions and pruritus, combined with the healing of existing lesions. Complete remission rates, relapse rates, changes in Bullous Pemphigoid Disease Area Index (BPDAI) scores, itching numerical rating scale (NRS) scores, laboratory results within 64 weeks, and adverse events (AEs) were also assessed.ResultsAmong 146 patients (median [IQR] age, 73 [64-85] years; 86 [58.9%] male patients) included in the study, 127 (87.0%) patients achieved disease control within 4 weeks, with a median (IQR) time of 14 (7-14) days. A total of 52 (35.6%) patients achieved complete remission, and 13 (8.9%) patients relapsed during the observation period. The complete remission rate and cumulative relapse rate at week 64 were 62.5% (5 of 8) and 30.9%, respectively. There was rapid and sustained improvement in clinical indicators and laboratory examination results after dupilumab treatment, including BPDAI scores, itching NRS scores, serum anti-BP180 and anti-BP230 antibodies, total IgE levels, and eosinophil count. Of these 146 patients, 107 (73.3%) did not report any AEs. The most common AEs were infections and eosinophilia. Serum anti-BP180 antibody levels of greater than 50 relative units (RU)/mL (OR, 3.63; 95% CI, 0.97-12.61; P = .045) were associated with 4-week disease control, and male patients were more likely to relapse (HR, 10.97; 95% CI, 1.42-84.92; P = .02).Conclusions and RelevanceIn this retrospective cohort study, dupilumab treatment was associated with improved clinical symptoms in patients with BP. The safety profile was favorable, although concurrent infection and eosinophilia might pose potential concerns. This study suggests that patients with anti-BP180 antibody levels of at least 50 RU/mL and female sex may respond better.

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Section: Discussionmentioning

confidence: 82%

Evaluation of Dupilumab in Patients With Bullous Pemphigoid

Zhao,

Wang,

Liang

et al. 2023

JAMA Dermatol

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“…85 Having both BP230-and BP180-IgG together with older age at onset have been associated with a favorable outcome, whereas, BP180 + /BP230serotypes and younger age are linked to a more severe disease with frequent mucosal involvement. 86,87 Finally, BP with antibodies against carboxy-terminal epitopes of BP180 show more a more commonly mucosal involvement. 86 Rarely, BP can be localized to limited areas of the skin.…”

Section: Bullous Pemphigoidmentioning

confidence: 99%

“…86,87 Finally, BP with antibodies against carboxy-terminal epitopes of BP180 show more a more commonly mucosal involvement. 86 Rarely, BP can be localized to limited areas of the skin. This variant is normally linked with the presence of triggers, such as bacterial infection, radiation therapy or surgical operation.…”

Section: Bullous Pemphigoidmentioning

confidence: 99%

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

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SummaryPemphigus and pemphigoid are two potentially life‐threatening groups of autoimmune diseases, characterized by autoantibodies targeting structural components of desmosomes or hemidesmosomes, respectively. Affected patients typically show itchy/painful plaques or blistering skin lesions and/or impairing mucosal blistering and erosions, which may strongly impact their quality of life. Since the milestone work of Walter Lever in 1953, who differentiated these two groups of diseases by histopathological analysis of the level of antibody‐mediated skin cleavage, enormous progresses occurred. Achievements made in laboratory diagnostics now allow to identify antigen specific structural proteins of the skin that are targeted by pathogenic autoantibodies. These progresses were accompanied by an increased understanding of the pathogenesis of these diseases thanks to the establishment of animal models reproducing disease and on studies on skin and blood of affected individuals, which have been leading to novel and disease‐specific treatments. Yet, given their phenotypical overlap with more common dermatological diseases, correct diagnosis and appropriate treatment are often delayed, in some cases leading to irreversible sequelae, including organ dysfunction (i.e., loss of vision in mucous membrane pemphigoid). Here, we provide a concise overview of the clinical appearance, diagnosis and therapeutic management of pemphigus and pemphigoid diseases.

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“…85 Das Vorliegen von BP230-und BP180-Antikörper sowie ein höheres Alter bei Krankheitsbeginn wird mit einem günstigeren Krankheitsverlauf in Verbindung gebracht, während BP180 + /BP230 --Serotypen und ein jüngeres Alter mit einer schwereren Erkrankung mit häufiger Schleimhautbeteiligung assoziiert sind. 86,87 Das BP mit Antikörper gegen carboxyterminale BP180-Epitope zeigt häufiger eine Schleimhautbeteiligung. 86 In seltenen Fällen kann ein BP auf einzelne Hautareale begrenzt sein.…”

Section: Bullöses Pemphigoidunclassified

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

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ZusammenfassungPemphigus und Pemphigoid sind seltene Autoimmunkrankheiten der Haut mit potenziell lebensbedrohlichem Verlauf. Autoantikörper gegen epidermale und junktionale Strukturproteine (Desmosomen sowie Hemidesmosomen) führen bei Betroffenen typischerweise zu juckenden, schmerzhaften Plaques oder Blasen an der Haut und/oder Blasenbildung und Erosionen der Schleimhäute mit möglicher Einschränkung der Lebensqualität. Seit der bahnbrechenden Arbeit von Walter Lever im Jahr 1953, dem es gelang, mittels histopathologischer Untersuchung diese beiden Krankheitsgruppen anhand des Musters der Antikörper‐vermittelten Blasenbildung zu differenzieren, wurden enorme Fortschritte im Verständnis der Erkrankungen erzielt. Die Errungenschaften in der Labordiagnostik ermöglichten die Identifikation von Zielstrukturen zur präzisen Unterscheidung verschiedener Varianten der bullösen Autoimmunerkrankungen. Diese Fortschritte gingen dank der Entwicklung von Tiermodellen mit einem besseren Verständnis der Pathogenese einher. Außerdem haben Studien an Haut und Blut betroffener Patienten zu neuen und krankheitsspezifischen Behandlungen geführt. Aufgrund ihrer Seltenheit und der klinischen Ähnlichkeit mit anderen dermatologischen Erkrankungen verzögern sich die korrekte Diagnosestellung und die Einleitung einer entsprechenden Therapie häufig, was in einigen Fällen zu irreversiblen Folgeerscheinungen, einschließlich Funktionsstörungen von Organen (zum Beispiel Verlust des Sehvermögens beim Schleimhautpemphigoid) führt. Wir geben hier einen Überblick über das klinische Erscheinungsbild, den Diagnosealgorithmus und das therapeutische Management von Pemphigus‐ und Pemphigoid‐Erkrankungen.

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Bullous pemphigoid: Three main clusters defining 3 outcome profiles

Cited by 7 publications

References 18 publications

Evaluation of Dupilumab in Patients With Bullous Pemphigoid

Evaluation of Dupilumab in Patients With Bullous Pemphigoid

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

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