Variation of the epidermal expression of glucocorticoid receptor‐beta as potential predictive marker of bullous pemphigoid outcome

Brulefert, Adrien; Jan, Sébastien Le; Plée, Julie; Durlach, A.; Bernard, Philippe; Antonicelli, Frank; Trussardi‐Régnier, Aurélie

doi:10.1111/exd.13444

Cited by 9 publications

(8 citation statements)

References 51 publications

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“…This is also in setting with the low prevalence of anti-type VII collagen antibodies in other autoimmune and autoinflammatory diseases, such as inflammatory bowel disease (16%) and pemphigus (9.5%), but also in healthy subjects ( 38 ). Furthermore, the low titer of anti-type VII collagen AAb in comparison with the high titer of anti-BP180 antibodies in our two patients with mucous membrane involvement, like in these other diseases, rather points out the production of anti-type VII collagen antibodies in BP as an epiphenomenon, as previously suggested ( 31 ). A possible diversification of the AAb response in BP could be related at least in part to disease severity, as BP patients with mucosal involvement had higher total and skin BPDAI scores, especially the blisters/erosions activity score, as compared with the BPDAI score from patients with a typical form of BP ( 41 ).…”

Section: Discussionsupporting

confidence: 75%

“…Actually, high serum level of anti-BP180 NC16A AAb was correlated with disease activity at time of diagnosis and was shown as an independent risk factor for BP relapse after cessation of therapy ( 14 , 15 , 25 – 27 ). In addition, approximately 30% of the BP patients relapse during the first year of treatment ( 16 , 28 – 31 ). For those latter BP patients, it was shown that the variations of serum IgG AAb directed toward the BP180 NC16A domain after 2 months of therapy may be useful to predict BP outcome ( 16 , 32 ).…”

Section: Introductionmentioning

confidence: 99%

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Anti-Type VII Collagen Antibodies Are Identified in a Subpopulation of Bullous Pemphigoid Patients With Relapse

et al. 2018

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Bullous pemphigoid (BP) is an autoimmune bullous skin disease characterized by anti-BP180 and anti-BP230 autoantibodies (AAbs). Mucous membrane involvement is an uncommon clinical feature of BP which may evoke epidermolysis bullosa acquisita, another skin autoimmune disease characterized by anti-type VII collagen AAbs. We therefore evaluated the presence of anti-type VII collagen AAbs in the serum of BP patients with and without mucosal lesions at time of diagnosis and under therapy. Anti-BP180, anti-BP230, and anti-type VII collagen AAbs were measured by ELISA in the serum of unselected patients fulfilling clinical and histo/immunopathological BP criteria at baseline (n = 71) and at time of relapse (n = 24). At baseline, anti-type VII collagen AAbs were detected in 2 out of 24 patients with BP presenting with mucosal involvement, but not in patients without mucosal lesions (n = 47). At the time of relapse, 10 out of 24 BP patients either displayed a significant induction or increase of concentrations of anti-type VII collagen AAbs (P < 0.01), independently of mucosal involvement. Those 10 relapsing BP patients were also characterized by a sustained high concentration of anti-BP180 AAb, whereas the serum anti-BP230 AAb concentrations did not vary in BP patients with relapse according to the presence of anti-type VII collagen AAbs. Thus, our study showed that anti-type VII collagen along with anti-BP180 AAbs detection stratified BP patients at time of relapse, illustrating a still dysregulated immune response that could reflect a potential epitope spreading mechanism in those BP patients.

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Section: Discussionsupporting

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Anti-Type VII Collagen Antibodies Are Identified in a Subpopulation of Bullous Pemphigoid Patients With Relapse

et al. 2018

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“…Moreover, an increased expression of the glucocorticoid receptor-beta in skin epithelial cells was suggested to be predictive of reduced treatment efficacy and increased risk of BP relapse (225). The presence of extensive disease (more than ten new blisters daily) at baseline and of neurological conditions associated with BP (e.g., dementia) may also play a role in the prediction of BP outcome (219).…”

Section: Predictive Biomarkers For Disease Progressionmentioning

confidence: 99%

Serological Diagnosis of Autoimmune Bullous Skin Diseases

Saschenbrecker¹,

Karl²,

Komorowski³

et al. 2019

Front. Immunol.

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Autoimmune bullous dermatoses (AIBD) encompass a variety of organ-specific autoimmune diseases that manifest with cutaneous and/or mucosal blisters and erosions. They are characterized by autoantibodies targeting structural proteins of the skin, which are responsible for the intercellular contact between epidermal keratinocytes and for adhesion of the basal keratinocytes to the dermis. The autoantibodies disrupt the adhesive functions, leading to splitting and blister formation. In pemphigus diseases, blisters form intraepidermally, whereas in all other disease types they occur subepidermally. Early identification of autoimmune bullous dermatoses is crucial for both treatment and prognosis, particularly as regards tumor-associated disease entities. The diagnosis is based on clinical symptoms, histopathology, direct immunofluorescence to detect antibody/complement deposits, and the determination of circulating autoantibodies. The identification of various target antigens has paved the way for the recent development of numerous specific autoantibody tests. In particular, optimized designer antigens and multiplex test formats for indirect immunofluorescence and ELISA have enhanced and refined the laboratory analysis, enabling highly efficient serodiagnosis and follow-up. This review elaborates on the current standards in the serological diagnostics for autoimmune bullous dermatoses.

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“…23,43 However, the refractory nature of some patients to corticosteroids merits further study. 44,45…”

Section: Bullous Pemphigoidmentioning

confidence: 99%

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

Santi

Gripp

Roselino

et al. 2019

An. Bras. Dermatol.

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Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.

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Variation of the epidermal expression of glucocorticoid receptor‐beta as potential predictive marker of bullous pemphigoid outcome

Cited by 9 publications

References 51 publications

Anti-Type VII Collagen Antibodies Are Identified in a Subpopulation of Bullous Pemphigoid Patients With Relapse

Anti-Type VII Collagen Antibodies Are Identified in a Subpopulation of Bullous Pemphigoid Patients With Relapse

Serological Diagnosis of Autoimmune Bullous Skin Diseases

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

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