Pemphigus Vulgaris

Shoimer, Ilya; Wong, Russell X.; Mydlarski, P. Régine

doi:10.1007/978-3-319-26728-9_1

Cited by 1 publication

(2 citation statements)

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“…The gold standard diagnostic of PV is direct immunofluorescence (DIF) microscopy, which can detect tissue-bound autoantibodies. In pemphigus, DIF microscopy reveals intercellular binding of IgG and/or C3 within the epidermis and/or epithelium (19).…”

Section: Diagnosismentioning

confidence: 99%

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Pemphigus vulgaris - approach and management (Review)

et al. 2019

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The place of pemphigus vulgaris (PV) among autoimmune bullous dermatoses is well known. In pemphigus, IgG autoantibodies are directed against desmogleins 1 and 3, which are part of the cadherin family of cell-cell adhesion molecules. These structures are responsible for maintaining the intercellular adherence in stratified squamous epithelia, such as the skin and oral mucosa. The incidence of autoimmune bullous dermatoses is steadily increasing, being associated with a high degree of morbidity. The pathophysiology of these dermatoses is very well understood, complemented by recent genetic studies. The gold standard for the diagnosis of pemphigus vulgaris is the detection of autoantibodies or complement component 3 by direct immunofluorescence microscopy of a perilesional biopsy. Early diagnosis and initiation of treatment are necessary in order to achieve a favorable prognosis. Although the first line of treatment is corticotherapy, there are no clear guidelines on dosing regimens, and long-term adverse effects are important. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. In addition, therapies with anti-CD20 antibodies are used, but antigen-specific immune suppression-based treatments represent the future.

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Section: Diagnosismentioning

confidence: 99%

“…Indirect immunofluorescence (IIF) is frequently used to semi-quantitatively measure circulating antibody levels, using monkey, rabbit or guinea pig esophagus as a substrate (19). The most frequently used substrates are monkey esophagus and human split skin.…”

Section: Diagnosismentioning

confidence: 99%