Updated French guidelines for the therapeutic management of bullous pemphigoid

Castel, M.; Alexandre, M.; Jelti, L.; Pham‐Ledard, Anne; Viguier, Manuelle; Bédane, C.; Tancrède‐Bohin, Emmanuelle; Musette, Philippe; Carvalho, P.; Cordel, N.; Caux, F.; Joly, P.

doi:10.1016/j.annder.2021.08.005

Cited by 15 publications

(9 citation statements)

References 45 publications

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“…This difference might be explained by a slower decrease in therapies that only began after disease control. Notably, topical CS was slowly tapered over 4 months, according to the French guidelines for the therapeutic management of BP ( 48 ). OMZ tapering was initiated only after CS weaning in all patients with CR.…”

Section: Discussionmentioning

confidence: 99%

Rapid Disease Control in First-Line Therapy-Resistant Mucous Membrane Pemphigoid and Bullous Pemphigoid with Omalizumab as Add-On Therapy: A Case Series Of 13 Patients

Alexandre¹,

Bohelay

Gille

et al. 2022

Front. Immunol.

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The role of IgE autoantibodies has been demonstrated in the pathogenesis of bullous pemphigoid for many years. Recently, omalizumab (OMZ), a humanized monoclonal anti-IgE antibody that depletes total serum IgE, has been used off-label in a few case series of bullous pemphigoids demonstrating a rapid efficacy and allowing significant improvements or complete remission as add-on therapy in first-line treatment-resistant patients. Herein, we report the largest retrospective study to evaluate OMZ effectiveness in patients with subepidermal autoimmune blistering diseases. Our series included 13 patients from a single center with bullous pemphigoid or mucous membrane pemphigoid, of whom 7 had mucous membrane involvement. OMZ was added to the unchanged immunosuppressive therapies. Detailed clinical and immunological data during the first year were collected, notably for specific anti-BP180-NC16A IgE and IgG, and the median total follow-up was 30 months (range: 3–81). Our series demonstrated that OMZ induced a significant improvement in pruritus, urticarial score, and daily blister count on day 15, allowing disease control to be achieved in a 1-month median time and complete remission (CR) in a 3-month median time in 85% of these patients previously in therapeutic impasse. At the end of the follow-up, 31% of patients achieved CR on minimal therapy after OMZ weaning without relapses, and 54% achieved CR on OMZ continuation with a minimal dose of concomitant treatment. Two patients experienced therapeutic failure (15%). At baseline, clinical variables reflecting activity were significantly positively correlated with eosinophil blood count, total IgE serum level, specific anti-BP180 IgE and IgG. While baseline anti-BP180 IgG and specific anti-BP180 IgE were significantly positively correlated, only the two patients who experienced a therapeutic failure with OMZ did not fit with this correlation, demonstrating elevated levels of anti-BP180 IgG with no measurable BP180-specific IgE. Follow-up of immunological variables demonstrated a rapid decrease of eosinophilia towards normalization, whereas a slower decline towards negativation was observed over 1 year for anti-BP180 IgG and anti BP180 IgE in patients who responded to OMZ. This case series demonstrated that OMZ is a rapidly effective biologic therapy for refractory bullous pemphigoid and mucous membrane pemphigoid, permitting rapid disease control and reduction of concomitant therapeutics.

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Section: Discussionmentioning

confidence: 99%

Rapid Disease Control in First-Line Therapy-Resistant Mucous Membrane Pemphigoid and Bullous Pemphigoid with Omalizumab as Add-On Therapy: A Case Series Of 13 Patients

Alexandre¹,

Bohelay

Gille

et al. 2022

Front. Immunol.

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“… 1 , 2 Complications have been found to arise secondary to treatment with systemic CS and immunosuppressive drugs, which are the mainstay of therapy. The latest European 10 and Italian 11 guidelines on the treatment of BP, described the primary goals of therapy as not only aimed at the treatment of the skin manifestations, pruritus and prevention of recurrences, but also underline the importance of improving the quality of life of patients, limiting the side‐effects related to the drugs employed, especially in the elderly.…”

Section: Discussionmentioning

confidence: 99%

“…Omalizumab, an anti‐IgE monoclonal antibody, has been included in the European, Italian, German 12 guidelines for patients with treatment‐resistant BP, with a Grade‐4 recommendation. Furthermore, it was recently cited in the updated French 2021 guidelines, 10 where it is listed as an option for the treatment of a subset of patients affected by corticosteroid‐dependent BP, an entity whose features preclude the possibility of gradually tapering the dosage of the systemic corticosteroids, thus exposing the patients to an increased risk of complications.…”

Section: Discussionmentioning

confidence: 99%

Omalizumab as a corticosteroid‐sparing agent in the treatment of bullous pemphigoid

Vassallo

Somenzi

Amici

et al. 2022

Dermatologic Therapy

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Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease, characterized by the development of autoantibodies against hemidesmosomal components BP180 and BP230. The mainstay of therapy is topical and systemic corticosteroids (CS) and immunosuppressors. As this pathology mainly involves the elderly, subjects often have numerous comorbidities that influence the clinical management. Omalizumab is a recombinant humanized monoclonal anti‐IgE antibody which has recently emerged as a promising treatment for BP in patients for whom CS are contraindicated or conventional treatments have failed to control the disease. For this study, we selected five patients who presented with corticosteroid‐dependent BP with a contraindication to the use of other immunosuppressive treatments. The objectives of our study were to evaluate the effectiveness of omalizumab in controlling BP and allowing to decrease the dosage of systemic CS, assessing the effects of omalizumab on the clinical manifestations and the titers of circulating anti‐BP180 and BP230 antibodies, IgE and eosinophils. A reduction in the dose of systemic CS was possible in 100% of the patients and complete resolution of the clinical picture was seen in 100% for skin lesions and in 40% for pruritus. A reduction of circulating IgE was found in 40%, anti‐BP180 and BP230 IgGs were decreased in 60% and eosinophils in 80%.

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“…Nonspecific activation of the immunity and the shared antigens between tumor cells and the BMZ have been involved in the development of PD-1/PD-L1 blockers-inducing BP (3,4,(68)(69)(70). In the treatment of BP, omalizumab, an antibody targeting IgE, and dupilumab, an antibody binding IL-4 receptor subunit a (IL-4Ra), are now increasingly utilized for the treatment of BP as novel therapeutic approaches (71)(72)(73).…”

Section: Other Biologics Inducing or Treating Bpmentioning

confidence: 99%

Paradoxical phenomena of bullous pemphigoid induced and treated by identical biologics

2023

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ObjectiveThis study aimed to investigate the clinical features of biologics-induced bullous pemphigoid (BP) and the therapeutic effects of those agents for BP, exploring the underlying pathophysiological mechanisms.MethodsWe searched PubMed, Web of Science, and Elsevier for studies involving pemphigoid patients treated with or induced by identical biologics published in English from January 2009 to April 2022.ResultsSeventeen cases of drug-induced BP associated with anti-tumor necrosis factor (aTNF)-α therapies, one with interleukin (IL)-17 inhibitors, and seven with IL-12/IL-23 or IL-23 inhibitors were enrolled. Time to cutaneous toxicity varied among different types of agents, and the characteristics of clinical examinations were similar to idiopathic BP. Discontinuation of the culprit drugs and initiation of topical or systemic corticosteroids were adequate in most cases. Several monoclonal antibodies above have also been reported for the treatment of refractory or recurrent BP, especially concurrent with psoriasis.ConclusionBiologics for immune-related diseases, including TNF-α, IL-17, and IL-12/IL-23 or IL-23 inhibitors, can both induce and treat BP, which might be associated with a helper T cells Th1/Th2 imbalance, complicated inflammatory networks, and a specific individual microenvironment, suggestive of a new perspective on the therapeutic algorithms of BP. There have been numerous reports about biologics inducing or treating BP. We have taken note of this phenomenon and focused on biologics with both pathogenetic and therapeutic effects on BP. Our review summarized the clinical characteristics of associated cases, trying to figure out the underlying mechanisms of this paradoxical phenomenon and to provide an integrated perspective and new therapeutic alternatives for BP.

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Updated French guidelines for the therapeutic management of bullous pemphigoid

Cited by 15 publications

References 45 publications

Rapid Disease Control in First-Line Therapy-Resistant Mucous Membrane Pemphigoid and Bullous Pemphigoid with Omalizumab as Add-On Therapy: A Case Series Of 13 Patients

Rapid Disease Control in First-Line Therapy-Resistant Mucous Membrane Pemphigoid and Bullous Pemphigoid with Omalizumab as Add-On Therapy: A Case Series Of 13 Patients

Omalizumab as a corticosteroid‐sparing agent in the treatment of bullous pemphigoid

Paradoxical phenomena of bullous pemphigoid induced and treated by identical biologics

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