Special Issue "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 2506

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

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Keywords

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

Published Papers (4 papers)

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Research

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Article
Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid
J. Clin. Med. 2021, 10(9), 1916; https://doi.org/10.3390/jcm10091916 - 28 Apr 2021
Cited by 2 | Viewed by 807
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
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Review

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Review
Use of Dupilumab in Bullous Pemphigoid: Where Are We Now?
J. Clin. Med. 2022, 11(12), 3367; https://doi.org/10.3390/jcm11123367 - 12 Jun 2022
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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
Review
A Review of the Immunologic Pathways Involved in Bullous Pemphigoid and Novel Therapeutic Targets
J. Clin. Med. 2022, 11(10), 2856; https://doi.org/10.3390/jcm11102856 - 18 May 2022
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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

Other

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Case Report
Sacral Dimple, Conjunctiva, and Nipple as Less Obvious Pemphigus Vulgaris Locations around Natural Body Orifices: A Report of Three Cases
J. Clin. Med. 2022, 11(10), 2855; https://doi.org/10.3390/jcm11102855 - 18 May 2022
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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
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