Emerging treatment options for the management of pemphigus vulgaris

Kridin, Khalaf

doi:10.2147/tcrm.s142471

Cited by 64 publications

(97 citation statements)

References 148 publications

(241 reference statements)

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“…Many other immunosuppressants, such as cyclophosphamide, dapsone, methotrexate, and intravenous immunoglobulin, have also been used in combination with prednisolone. A recent review comprehensively discusses medications used in PV and recommended treatment strategies (23).…”

Section: Discussionmentioning

confidence: 99%

Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland

Försti

Vuorre²,

Laurila³

et al. 2019

Acta Derm Venerol

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Pemphigus comprises a group of autoimmune skin diseases characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe, but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. To the best of our knowledge, there are no recent reports of the incidence of pemphigus in Nordic populations. The latest study into the incidence of pemphigus in Finland is from the 1970s. However, the incidence may have been affected, for example, by immigration. This is a retrospective database study of all patients with pemphigus diagnosed in the Oulu University Hospital between 1985 and 2017. The current study included 46 patients, and interestingly, the most prevalent subtype was superficial pemphigus foliaceus or erythematosus (65%). This study also reports comorbidities of study patients and treatments used for pemphigus in the Oulu University Hospital. Pemphigus is an autoimmune skin disease characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe, but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. The aim of this study was to investigate the incidence of pemphigus subtypes in Northern Finland between 1985 and 2017. A total of 46 patients diagnosed with pemphigus at the Department of Dermatology of Oulu University Hospital were found; the female/male ratio was 1.7. In contrast to many other countries, it was found that, in Northern Finland, the superficial pemphigus subtypes were the most common: erythematosus or foliaceus (65%) followed by pemphigus vulgaris (26%). Over the past 4 decades the annual incidence of pemphigus in Finland has increased from 0.76 to 2.8 cases per million persons.

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Section: Discussionmentioning

confidence: 99%

Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland

Försti

Vuorre²,

Laurila³

et al. 2019

Acta Derm Venerol

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“…However, before the introduction of systemic corticosteroids, most of the patients died within 2 years after diagnosis. With the development of corticosteroids and additional adjuvant therapies over the last decades, a significant reduction in the morbidity and mortality rates has been achieved [4,5].…”

Section: Introductionmentioning

confidence: 99%

The role of pentraxin 3 in pemphigus vulgaris

Yavuz

2020

pdia

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Introduction: Pemphigus is a group of autoimmune bullous diseases caused by antibodies directed against the desmosomal adhesion molecules desmoglein 1 and 3, which are required for intercellular adhesion of keratinocytes. Pentraxins are a group of proteins that function as pattern recognition molecules and also play a role in humoral innate immunity. Pentraxin 3 (PTX3) is the prototype of the long pentraxins and has been shown to be increased in numerous autoimmune diseases. Aim: To investigate whether PTX3 can be used as a marker of PV caused by autoimmunity and resulting in tissue injury. Material and methods: The study included 30 patients who presented to the University Medical School Dermatology Department and were diagnosed with PV based on clinical, histological, and immunological findings. The control group included 30 healthy individuals. Human PTX3 concentration was measured with a commercially available ELISA kit, using a double antibody sandwich enzyme-linked immunosorbent assay. Results: The 60 participants comprised 31 (52%) men and 29 (48%) women. The most common site of onset was mucosa + skin (n = 22; 73.3%) and a psychological pathology was present in 7 (23.3%) patients. Median PTX3 level was significantly higher in the PV group compared to the control group (p = 0.008). The ROC curve analysis indicated a significant area under curve (AUC) value for serum PTX3 level in the prediction of PV. Conclusions: PTX3 was found to be increased in PV and PTX3 could be a useful indicator of disease activity in PV.

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“…Infections, osteoporosis and metabolic derangements are frequently encountered adverse effects secondary to therapy . Numerous advances in our understanding of these diseases have allowed for treatment strategies that go beyond non‐specific immunosuppression . The application of rituximab and intravenous immunoglobulin (IVIg) in combination has shown how different aspects of these diseases can be effectively targeted .…”

Section: Introductionmentioning

confidence: 99%

“…16,18,[28][29][30][31][32][33] Numerous advances in our understanding of these diseases have allowed for treatment strategies that go beyond non-specific immunosuppression. 34,35 The application of rituximab and intravenous immunoglobulin (IVIg) in combination has shown how different aspects of these diseases can be effectively targeted. [36][37][38][39][40][41] While these therapies have become more accepted as a standard of care, corticosteroids are still generally required for prolonged periods of time for acute disease.…”

Section: Introductionmentioning

confidence: 99%

From bench to bedside: evolving therapeutic targets in autoimmune blistering disease

Kridin

Kowalski

Kneiber

et al. 2019

Acad Dermatol Venereol

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Autoimmune blistering diseases comprise a group of heterogenous conditions characterized by the loss of tolerance and subsequent development of autoantibodies targeting epidermal and subepidermal adhesion proteins. Blisters and erosions form on the skin and mucous membranes leading to significant morbidity and mortality. Traditional therapies rely on systemic immunosuppression. Advancements in our understanding of the pathophysiology of pemphigus and pemphigoid have led to the development of molecules which target specific pathways involved in induction and perpetuation of disease. In this review, we outline the novel therapeutic strategies including B‐cell depletion, T‐regulatory cell repletion, cell signalling inhibitors and small molecular inhibitors, inhibitory monoclonal antibodies, as well as complement inhibition. We additionally review their current level of clinical evidence. We lastly review therapeutics targets gleaned from the experimental epidermolysis bullosa acquisita mouse model. These emerging treatments offer an exciting progression from basic science discoveries that have the potential to transform the treatment paradigm in autoimmune blistering diseases.

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Emerging treatment options for the management of pemphigus vulgaris

Cited by 64 publications

References 148 publications

Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland

Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland

The role of pentraxin 3 in pemphigus vulgaris

From bench to bedside: evolving therapeutic targets in autoimmune blistering disease

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