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Pemphigus
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=== Treatment options === * Topical steroids, such as clobetasol * Intralesional injection of steroids, such as [[dexamethasone]] * Immunosuppressant drugs, such as CellCept ([[mycophenolic acid]]): In recent years, adjuvant drugs, especially biologics, have shown great promise.<ref>[http://www.bad.org.uk/public/leaflets/bad_patient_information_gateway_leaflets/pemphigus/treated/steroid.asp British Association of Dermtologists, Steroid sparing (or adjuvant) drugs] {{Webarchive|url=https://web.archive.org/web/20080918083600/http://www.bad.org.uk/public/leaflets/bad_patient_information_gateway_leaflets/pemphigus/treated/steroid.asp |date=2008-09-18 }}</ref> * Serum- or plasma-pooled products, such as intravenous [[gamma globulin]] (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus. * Biologics such as [[Rituximab]], an anti-[[CD20]] antibody, which was found to improve otherwise severe cases of recalcitrant pemphigus vulgaris.<ref name="pmid17065638">{{cite journal|vauthors=Ahmed AR, Spigelman Z, Cavacini LA, Posner MR |title=Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin|journal= New England Journal of Medicine|volume=355|issue=17|pages=1772β9|year=2006|pmid=17065638|doi=10.1056/NEJMoa062930 |doi-access=free}}</ref><ref name="pmid17687130">{{cite journal |vauthors=Joly P, Mouquet H, Roujeau JC, etal |title=A single cycle of rituximab for the treatment of severe pemphigus|journal= New England Journal of Medicine|volume=357|issue=6|pages=545β52|year=2007|pmid=17687130|doi=10.1056/NEJMoa067752|doi-access=free}}</ref> Currently rituximab is considered a first-line treatment for some pemphigus patients according to Delphi consensus recommendations <ref name="pmid29438767">{{cite journal|vauthors=Murrell DF et al | title=Diagnosis and management of pemphigus: Recommendations of an international panel of experts|journal= Journal of the American Academy of Dermatology| volume=82|issue=3|pages=575β585|year=2020|pmid=29438767|doi=10.1016/j.jaad.2018.02.021| pmc=7313440}}</ref> All of these drugs may cause severe side effects, so patients should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects. A meta-analysis of the literature found insufficient evidence to determine the optimal treatment regimen for pemphigus vulgaris and pemphigus foliaceus, but it found that adding [[Cyclophosphamide|cyclophosphamid]] and [[azathioprine]] to a glucocorticoid regimen reduced the amount of glucocorticoid needed for treatment, and topical epidermal growth factor significantly reduced lesion healing time.<ref>{{Cite journal|last1=Martin|first1=Linda K|last2=Agero|first2=Anna Liza|last3=Werth|first3=Victoria|last4=Villanueva|first4=Elmer|last5=Segall|first5=Janet|last6=Murrell|first6=Dedee F|date=2009-01-21|title=Interventions for pemphigus vulgaris and pemphigus foliaceus|url=http://www.cochrane.org/CD006263/SKIN_interventions-for-pemphigus-vulgaris-and-pemphigus-foliaceus|journal=Cochrane Database of Systematic Reviews|issue=1|pages=CD006263|language=en|doi=10.1002/14651858.CD006263.pub2|pmid=19160272|s2cid=34912494 |issn=1465-1858|url-access=subscription}}</ref> If skin lesions do become infected, [[antibiotic]]s may be prescribed. Tetracycline antibiotics have a mildly beneficial effect on the disease and are sometimes enough for pemphigus foliaceus. In addition, [[talcum powder]] is helpful to prevent oozing sores from adhering to bedsheets and clothes. Wound care and treatments are often akin to those used in burn units, including careful use of dressings that don't stick to the wounds, etc. If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immunosuppressant drugs is sometimes used in an attempt to halt the rapid progression of [[bronchiolitis obliterans]], including [[methylprednisolone]], [[ciclosporin]], [[azathioprine]], and [[thalidomide]]. [[Plasmapheresis]] may also be useful. New research shows that the treatment options and clinical management of pemphigus are advancing, and new [[B cell|B-cell]][[Targeted therapy|-targeted-therapies]] are beginning to replace the broad-spectrum [[immunosuppression]] treatments.<ref>Lim, Y. L., Bohelay, G., Hanakawa, S., Musette, P., & Janela, B. (2022). Autoimmune pemphigus: latest advances and emerging therapies. Frontiers in Molecular Biosciences, 8. https://doi.org/10.3389/fmolb.2021.808536</ref> While promising, these new therapies may have significant side effects; hence, supporting research and extensive trials are needed to verify their effectiveness and safety.<ref>Lim, Y. L., Bohelay, G., Hanakawa, S., Musette, P., & Janela, B. (2022). Autoimmune pemphigus: latest advances and emerging therapies. Frontiers in Molecular Biosciences, 8. https://doi.org/10.3389/fmolb.2021.808536</ref>
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