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Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders
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Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Dermatology".

Deadline for manuscript submissions: closed (31 May 2022) | Viewed by 22546

Special Issue Editor

Prof. Dr. Aikaterini Patsatsi

E-Mail Website
Guest Editor
2nd Department of Dermatology, Αutoimmune Bullous Diseases Unit, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece
Interests: autoimmune bullous diseases; lupus; dermatomyositis; cutaneous lymphomas; psoriasis; atopic dermatitis

Special Issue Information

Dear Colleagues,

Autoimmune bullous diseases are typical examples of autoantibody-mediated, organ-specific autoimmune disorders. Although there are still gaps in the precise description of their pathogenetic mechanisms, we know that the key step is the formation of circulating and tissue-bound autoantibodies against target adhesion structures of the skin and mucous membranes.

This spectrum of diseases was included in the spectrum of orphan diseases for decades. After the introduction of systemic steroids, most of the available immunomodulatory additive medications were not able to achieve a significant and durable therapeutic response.

Rituximab was a huge step in the treatment of pemphigus, and many more targeted therapies have been investigated in pemphigus, bullous pemphigoid and other less frequent autoimmune bullous diseases.

In this issue, we expect to present information on the emerging therapies in this extremely interesting field of dermatology.

Prof. Dr. Aikaterini Patsatsi
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • Bullous pemphigoid
  • Epidermolysis bullosa acquisita
  • Mucous membrane pemphigoid
  • Dermatitis herpetiformis

Published Papers (7 papers)

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Research

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6 pages, 230 KiB  
Article
Rituximab in Mucous Membrane Pemphigoid: A Monocentric Retrospective Study in 10 Patients with Severe/Refractory Disease
by Maria Efenesia Baffa, Alberto Corrà, Roberto Maglie, Elena Biancamaria Mariotti, Francesca Montefusco, Carlo Pipitò, Stefano Senatore, Lavinia Quintarelli, Marzia Caproni and Emiliano Antiga
J. Clin. Med. 2022, 11(14), 4102; https://doi.org/10.3390/jcm11144102 - 15 Jul 2022
Cited by 2 | Viewed by 1350
Abstract
Rituximab (RTX) is a monoclonal antibody directed against CD20 antigen indicated in an increasing number of immune-mediated diseases. While its efficacy in pemphigus vulgaris has been widely investigated, only a few data about its possible role in pemphigoid diseases have been reported in [...] Read more.
Rituximab (RTX) is a monoclonal antibody directed against CD20 antigen indicated in an increasing number of immune-mediated diseases. While its efficacy in pemphigus vulgaris has been widely investigated, only a few data about its possible role in pemphigoid diseases have been reported in the literature. Accordingly, herein we evaluated a case series of patients with mucous membrane pemphigoid (MMP) treated with RTX. We included patients with a history of severe/refractory MMP who received at least one cycle of intravenous RTX between May 2018 and December 2021 and had 6 months of follow-up time. Disease control (DC) was our early endpoint, while complete remission (CR) and partial remission (PR) were late endpoints. CR off-therapy, relapses, and adverse events were evaluated as well. Our population included 10 MMP patients. Eight out of ten patients (80%) achieved DC in a mean of 8 weeks, while two patients with ocular MMP were non-responders. Among the eight patients who achieved DC, two reached CR off therapy, two CR on minimal therapy, and two achieved PR on minimal therapy. In our case series, the addition of RTX to conventional therapies was demonstrated to be safe and effective in reaching rapid disease control in the majority of refractory MMP patients. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
12 pages, 1467 KiB  
Article
Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid
by Ágnes Kinyó, Anita Hanyecz, Zsuzsanna Lengyel, Dalma Várszegi, Péter Oláh, Csaba Gyömörei, Endre Kálmán, Tímea Berki and Rolland Gyulai
J. Clin. Med. 2021, 10(9), 1916; https://doi.org/10.3390/jcm10091916 - 28 Apr 2021
Cited by 10 | Viewed by 2245
Abstract
Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our [...] Read more.
Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our aim was to assess DPP4i-induced bullous pemphigoid among our BP patients and characterize the clinical, laboratory and histological features of this drug-induced disease form. In our patient cohort, out of 127 BP patients (79 females (62.2%), 48 males (37.7%)), 14 (9 females and 5 males) were treated with DPP4i at the time of BP diagnosis. The Bullous Pemphigoid Disease Area Index (BPDAI) urticaria/erythema score was significantly lower, and the BPDAI damage score was significantly higher in DPP4i-BP patients compared to the nonDPP4i group. Both the mean absolute eosinophil number and the mean periblister eosinophil number was significantly lower in DPP4i-BP patients than in nonDPP4i cases (317.7 ± 0.204 vs. 894.0 ± 1.171 cells/μL, p < 0.0001; 6.75 ± 1.72 vs. 19.09 ± 3.1, p = 0.0012, respectively). Our results provide further evidence that DPP4i-associated BP differs significantly from classical BP, and presents with less distributed skin symptoms, mild erythema, normal or slightly elevated peripheral eosinophil count, and lower titers of BP180 autoantibodies. To our knowledge, this is the first case series of DPP4i-related BP with a non-inflammatory phenotype in European patients. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
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Review

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17 pages, 1163 KiB  
Review
Epidermolysis Bullosa Acquisita—Current and Emerging Treatments
by Deša Tešanović Perković, Zrinka Bukvić Mokos and Branka Marinović
J. Clin. Med. 2023, 12(3), 1139; https://doi.org/10.3390/jcm12031139 - 1 Feb 2023
Cited by 1 | Viewed by 2923
Abstract
Epidermolysis bullosa acquisita (EBA) is a rare chronic autoimmune subepidermal blistering disease of the skin and mucous membranes, usually beginning in adulthood. EBA is induced by autoantibodies to type VII collagen, a major component of anchoring fibrils in the dermal–epidermal junction (DEJ). The [...] Read more.
Epidermolysis bullosa acquisita (EBA) is a rare chronic autoimmune subepidermal blistering disease of the skin and mucous membranes, usually beginning in adulthood. EBA is induced by autoantibodies to type VII collagen, a major component of anchoring fibrils in the dermal–epidermal junction (DEJ). The binding of autoantibodies to type-VII collagen subsequently leads to the detachment of the epidermis and the formation of mucocutaneous blisters. EBA has two major clinical subtypes: the mechanobullous and inflammatory variants. The classic mechanobullous variant presentation consists of skin fragility, bullae with minimal clinical or histological inflammation, erosions in acral distribution that heal with scarring, and milia formation. The inflammatory variant is challenging to differentiate from other autoimmune bullous diseases, most commonly bullous pemphigoid (BP) but also mucous membrane pemphigoid (MMP), Brunsting–Perry pemphigoid, and linear IgA dermatosis. Due to its recalcitrance conventional treatment of epidermolysis bullosa acquisita is shown to be demanding. Here we discuss novel therapeutic strategies that have emerged and which could potentially improve the quality of life in patients with EBA. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
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14 pages, 1169 KiB  
Review
Pemphigus during the COVID-19 Epidemic: Infection Risk, Vaccine Responses and Management Strategies
by Xueyi Huang, Xiaoqian Liang, Jiao Zhang, Hang Su and Yongfeng Chen
J. Clin. Med. 2022, 11(14), 3968; https://doi.org/10.3390/jcm11143968 - 8 Jul 2022
Cited by 5 | Viewed by 2467
Abstract
Pemphigus is a rare autoimmune blistering disease, involving potentially life-threatening conditions often requiring immunosuppression. Currently, the COVID-19 pandemic caused by severe acute respiratory disease coronavirus 2 (SARS-CoV-2) infection has become a global public emergency. Vaccines are the most effective defense against COVID-19 infection. [...] Read more.
Pemphigus is a rare autoimmune blistering disease, involving potentially life-threatening conditions often requiring immunosuppression. Currently, the COVID-19 pandemic caused by severe acute respiratory disease coronavirus 2 (SARS-CoV-2) infection has become a global public emergency. Vaccines are the most effective defense against COVID-19 infection. However, in clinic, there are cases of new onset or flare of pemphigus following COVID-19 vaccination, where vaccines have manifested significantly desirable risk-benefit profiles for patients. Although Rituximab, as first-line therapy, may impair humoral immunity, pemphigus may not predispose to develop COVID-19 infection compared to a healthy population. Conversely, delay or interruption of immunosuppressants probably results in unfavorable clinical outcomes for disease progression. Overall, clinicians should encourage their patients to undergo the vaccination after a comprehensive assessment. The definite association between COVID-19 vaccination and pemphigus remains to be further elucidated. Herein, we provide an overview of the published studies to date on COVID-19 and pemphigus as well as the exploration of their complicated interplay. In addition, we discuss the management strategies for pemphigus patients in this special period, in an effort to more effectively establish a standard treatment paradigm for this particular patient group. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
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7 pages, 210 KiB  
Review
Use of Dupilumab in Bullous Pemphigoid: Where Are We Now?
by Roberto Russo, Niccolò Capurro, Emanuele Cozzani and Aurora Parodi
J. Clin. Med. 2022, 11(12), 3367; https://doi.org/10.3390/jcm11123367 - 12 Jun 2022
Cited by 9 | Viewed by 3125
Abstract
Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal bullous disease. At present, the main treatment options are represented by corticosteroids and immunosuppressant drugs. Steroids often need to be administered in high doses, with subsequent adverse events and safety issues, as BP mainly [...] Read more.
Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal bullous disease. At present, the main treatment options are represented by corticosteroids and immunosuppressant drugs. Steroids often need to be administered in high doses, with subsequent adverse events and safety issues, as BP mainly affects elderly people. As dupilumab, a recombinant fully human IgG4 monoclonal antibody with binding specificity to human interleukin-4 receptor IL-4Rα has become paramount in the treatment of atopic dermatitis, its use in autoimmune bullous diseases has been theorized and it has been used to treat patients with BP. Dupilumab seems to be an effective and safe option to treat recalcitrant BP. Here, we report the results of a literature review on the use of dupilumab in BP, including a total of 30 treated patients in 9 papers. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
12 pages, 292 KiB  
Review
A Review of the Immunologic Pathways Involved in Bullous Pemphigoid and Novel Therapeutic Targets
by Mohsen Afarideh, Robert Borucki and Victoria P. Werth
J. Clin. Med. 2022, 11(10), 2856; https://doi.org/10.3390/jcm11102856 - 18 May 2022
Cited by 10 | Viewed by 2317
Abstract
Bullous pemphigoid (BP) is a rare, chronic antibody-mediated autoimmune blistering disease primarily affecting the elderly, with an age of onset over 60. Current treatment options are limited and involve the use of corticosteroids and immunosuppressants, but their long-term use is associated with significant [...] Read more.
Bullous pemphigoid (BP) is a rare, chronic antibody-mediated autoimmune blistering disease primarily affecting the elderly, with an age of onset over 60. Current treatment options are limited and involve the use of corticosteroids and immunosuppressants, but their long-term use is associated with significant morbidity and mortality. In Japan, human intravenous immunoglobin is approved for the treatment of corticosteroid-refractory BP. However, no treatment option is approved by the Food and Drug Administration for the management of BP. Therefore, developing effective therapies free of debilitating side effects is imperative. In this review, we summarize the main immunologic pathways involved in the pathogenesis of BP, with an emphasis on the role of eosinophils, immunoglobulins, cytokines such as the interleukin (IL)-4 and IL-5, and complements. We further discuss the latest advances with novel therapeutic targets tested for the management of BP. Ongoing efforts are needed to run well-designed controlled trials and test the efficacy and safety of investigational drugs while providing much-needed access to these medications for refractory patients who will not otherwise be able to afford them as off-label prescriptions. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)

Other

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10 pages, 1503 KiB  
Case Report
Sacral Dimple, Conjunctiva, and Nipple as Less Obvious Pemphigus Vulgaris Locations around Natural Body Orifices: A Report of Three Cases
by Magdalena Jałowska, Justyna Gornowicz-Porowska, Monika Bowszyc-Dmochowska and Marian Dmochowski
J. Clin. Med. 2022, 11(10), 2855; https://doi.org/10.3390/jcm11102855 - 18 May 2022
Cited by 7 | Viewed by 7207
Abstract
In this paper, we present our own clinical-laboratory experience concerning three less obvious presentations of pemphigus vulgaris (PV) and discuss the pertinent literature. The involvement of the sacral dimple reported here for the first time, as well as the nipple and the eyes, [...] Read more.
In this paper, we present our own clinical-laboratory experience concerning three less obvious presentations of pemphigus vulgaris (PV) and discuss the pertinent literature. The involvement of the sacral dimple reported here for the first time, as well as the nipple and the eyes, could initially be misleading clinically. These less stereotypical localizations may occur due to the transition of different epithelia, each with varying levels of cadherin (desmoglein, desmocollin) and thus altered sensitivity to mechanical stress. The role of dermatologists who have experience in treating autoimmune blistering dermatoses is fundamental for identifying promptly the initial and exacerbating PV lesions in such unusual locations. Full article
(This article belongs to the Special Issue Current Concept and Emerging Treatments in Autoimmune Bullous Skin Disorders)
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