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Pemphigus Vulgaris
Published in Charles Theisler, Adjuvant Medical Care, 2023
Pemphigus is a group of rare autoimmune disorders that causes thin walled, flaccid, easily-ruptured blisters and sores on the top layer of skin and mucous membranes, such as in the mouth, throat, nose, and genitals. It is an autoimmune disorder and is not contagious. The disease occurs almost exclusively in middle-aged or older people and tends to be a long-lasting chronic condition. The type of pemphigus diagnosed depends on where the blisters form. Pemphigus vulgaris with blisters on the skin and in the mouth is the most common type of pemphigus. Wherever the blisters form, they tend to break open quickly, leaving painful sores. The disorder can sometimes be cause by certain medications such as ACE inhibitors, penicillamine, cephalosporin, pyrazolones, NSAIDs, and rifampin.1 The goals of treatment are to promote and maintain remission, as well as to avoid complications, such as infections.1
Blistering diseases in the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Pemphigus is a rare blistering disease that affects the skin and the mucous membranes; it has many variants, the vulgaris form being most common1. Less common ones include vegetans, foliaceus, erythematosus, drug-induced and fogo selvagem2,3. Pemphigus vulgaris affects both genders equally, with the majority age of presentation being 50–60 years old3. The worldwide incidence is very low, with a higher incidence seen in Ashkenazi Jews1.
Novel Treatments of Autoimmune Conditions
Published in Irun R. Cohen, Perspectives on Autoimmunity, 2020
Y. Shoenfeld, Y. Tomer, O. Ben-Yehuda
Circulating autoantibodies reacting to epidermal intercellular antigens are believed to play a role in the pathogenesis of pemphigus.21 In most trials of PE in pemphigus the symptoms greatly improved, and the doses of corticosteroids or immunosuppressive drugs required to control the disease were reduced substantially.21,22 There are also case reports in which PE was successfully used to treat insulin resistance diabetes mellitus resulting from development of antiinsulin autoantibodies,23 and red cell aplasia due to autoantibodies directed against erythropoietic progenitors.24
Autoimmune blistering diseases: promising agents in clinical trials
Published in Expert Opinion on Investigational Drugs, 2023
Henning Olbrich, Christian D. Sadik, Enno Schmidt
Pemphigus diseases are treated systemically unless the disease is clinically very limited. Initial therapy for mild pemphigus includes systemic steroids (1–1.5 mg/kg/day prednisolone) and adjuvant azathioprine, mycophenolate or, only for PF, dapsone. With this regimen, about 20–30% of patients achieve complete remission [39]. Alternatively, rituximab can be given. Severe disease is preferentially treated with rituximab as the first-line treatment, initially combined with systemic corticosteroids and possibly immunosuppressants. In this approach, about 25% of pemphigus patients will suffer from a relapse and 40% from severe adverse events [40]. Refractory cases can receive higher corticosteroid doses, adjuvant immunoadsorption, or IVIG. In all stages, antiseptic measures are recommended and topical corticosteroids or calcineurin inhibitors can be given [26,31,33].
Prospects for CAR T cell immunotherapy in autoimmune diseases: clues from Lupus
Published in Expert Opinion on Biological Therapy, 2022
Marko Radic, Indira Neeli, Tony Marion
Certain autoimmune diseases result from the effects of a single autoantibody specificity. Pemphigus is an autoimmune skin blistering disorder, in which autoantibodies play a central role. Most patients with pemphigus exhibit autoantibodies to desmoglein, a cadherin-like adhesion molecule that connects keratinocytes to each other and gives elasticity and cohesion to the epidermis [71]. Ellebrecht et al. [72] constructed CARs with extracellular domains derived from desmoglein to serve as ‘bait’ for anti-desmoglein B cells (Figure 3B). Upon binding to anti-desmoglein B cells, the redesigned chimeric autoantibody receptor (CAAR) triggers the specific killing of the autoreactive B cells. The success of this approach in animals provided preclinical data used in an application to the FDA for the initiation of clinical trials [73]. A similar approach has been developed for myasthenia gravis (MG), a progressive and debilitating disease with autoimmune disruption of acetylcholine receptor function at the neuromuscular junctions. A subset of patients with MG make autoantibodies to muscle signaling kinase (MuSK) and CAAR T cells using a portion of MuSK as the extracellular bait for autoreactive B cells are in early development (Oh S, 2020). Yet to be tested in the context of autoimmunity, a CAR T cell incorporating domains from a scavenging protein (Figure 3C) could offer an alternative approach to deplete excessive circulating IC, as shown by engineering in a phagocytic cell population [74].
Comparing the short-term therapeutic effects and safety profiles of rituximab therapy in pemphigus vulgaris patients either early treated or later than six months
Published in Journal of Dermatological Treatment, 2019
Kamran Balighi, Maryam Daneshpazhooh, Zahra Akbari, Soheil Tavakolpour, Pourya Azimi, Arghavan Azizpour
Various interventions have been used to treat pemphigus. However, the optimal therapeutic strategy has not yet been established. High-dose, long-term corticosteroids, or immune-suppressants are the cornerstone of the management of pemphigus. Unfortunately, in spite of effeteness of these drugs, adverse effects of long-term treatment with such agents have emerged as the primary causes of morbidity and mortality in pemphigus patients (3,4). In view of these side effects, alternatives for the long-term management of pemphigus have been sought. Increasing evidence supports the use of rituximab in the treatment of pemphigus, as an adjuvant agent or after other treatment modalities have failed and, more recently, as a first-line treatment (5). Rituximab is a chimeric monoclonal IgG against CD20 on pre- and mature B cells; it is currently used off-label for dermatological disorders, such as chronic graft versus host disease, collagen vascular diseases, and autoimmune bullous dermatoses (6,7). However, there is currently no consensus regarding the guidelines on the use of this agent in PV patients (8,9).