Treatment of Pemphigus Vulgaris and Pemphigus Foliaceus: A Systematic Review and Meta-Analysis

Atzmony, Lihi; Hodak, Emmilia; Gdalevich, Michael; Rosenbaum, Omer; Mimouni, Daniel

doi:10.1007/s40257-014-0101-9

Cited by 33 publications

(29 citation statements)

References 31 publications

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“…We also observed that patients with PV with mucocutaneous symptoms had the lowest EQ‐5D‐5L index scores, which highlights the importance of additional supportive treatments for patients with mucosal symptoms (e.g. topical corticosteroids, gels containing local anaesthetics and intralesional corticosteroid injections) …”

Section: Discussionmentioning

confidence: 58%

Validity of the EQ ‐5D in patients with pemphigus vulgaris and pemphigus foliaceus

et al. 2018

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Section: Discussionmentioning

confidence: 58%

Validity of the EQ ‐5D in patients with pemphigus vulgaris and pemphigus foliaceus

et al. 2018

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“…Published guidelines for pemphigus therapy mostly rely on expert consensus 129–131 , given the paucity of randomized clinical trials with large sample sizes and rigorous randomization methods 132 .…”

Section: Managementmentioning

confidence: 99%

Pemphigus

Kasperkiewicz

Ellebrecht

Takahashi

et al. 2017

Nat Rev Dis Primers

427

429

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Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell–cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms. The pathophysiological roles of T cells and B cells have been characterized in mouse models of pemphigus and patients, revealing insights into the mechanisms of autoimmunity. Diagnosis is based on clinical manifestations and confirmed with histological and immunochemical testing. The current first-line treatment is systemic corticosteroids and adjuvant therapies, including immunosuppressive agents, intravenous immunoglobulin and plasmapheresis. Rituximab, a monoclonal antibody against CD20+ B cells, is a promising therapeutic option that may soon become first-line therapy. Pemphigus is one of the best-characterized human autoimmune diseases and provides an ideal paradigm for both basic and clinical research, especially towards the development of antigen-specific immune suppression treatments for autoimmune diseases.

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“…The superiority of one adjuvant has not been confirmed by trials. 59,60 PV and PF are the only conditions for which dermatological RCTs evaluating cyclophosphamide efficacy and safety have been performed.…”

Section: Pv and Pemphigus Foliaceus (Pf)mentioning

confidence: 99%

Cyclophosphamide in dermatology

Kim

Chan

2016

Aust J Dermatology

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Cyclophosphamide is a chemotherapeutic agent which was first discovered in experimental tumours in rats, and it has since been widely used to treat malignancies and severe manifestations of various auto-immune diseases. High-dose chemotherapy and continuous daily oral regimens are associated with significant toxicity profiles, but i.v. pulsed regimens have lowered the rates of adverse effects in rheumatological studies. Cyclophosphamide has been shown to be useful in the treatment of severe autoimmune conditions due to its powerful immunosuppressive ability; however, it remains a relatively underused modality in dermatology. This article reviews the current literature on cyclophosphamide and its clinical applications in dermatology.

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Treatment of Pemphigus Vulgaris and Pemphigus Foliaceus: A Systematic Review and Meta-Analysis

Cited by 33 publications

References 31 publications

Validity of the EQ ‐5D in patients with pemphigus vulgaris and pemphigus foliaceus

Validity of the EQ ‐5D in patients with pemphigus vulgaris and pemphigus foliaceus

Pemphigus

Cyclophosphamide in dermatology

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