Pemphigus by Ray Sahelian, M.D.
Pemphigus vulgaris is a life-threatening autoimmune blistering skin disease. Pemphigus is an autoimmune skin disease that can present in a variety of forms and can prove challenging to manage and treat. Mortality is high without treatment. See a list of autoimmune diseases.
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Treatment of Pemphigus vulgaris
The most commonly used treatments are (glucocorticoids,
azathioprine), the second line therapies (cyclosporine and mycophenolate mofetil),
and additional alternative treatments (cyclophosphamide and dapsone). The
combination of rituximab and intravenous immune globulin is effective in
patients with refractory pemphigus vulgaris.
Pemphigus is
usually treated with high-dose corticosteroids in combination with other immunosuppressants. However, this regimen may prove inadequate in severe cases
and can cause dangerous side-effects. Protein A immunoadsorption (PAIA) may be an effective adjuvant treatment for induction of
remission in severe pemphigus. However, in a significant number of cases, the
disease rapidly recurs once PAIA and immunosuppressive medication are
tapered.
In one study regarding the treatment of pemphigus vulgaris, the efficacy
of prednisolone was enhanced when it was combined with a cytotoxic drug. The
most efficacious cytotoxic drug to reduce steroid use was found to be
azathioprine, followed by cyclophosphamide (pulse therapy), and mycophenolate
mofetil.
Phenol induced Pemphigus
A possible mechanism for phenol-induced pemphigus lesions in genetically predisposed individuals is proposed that accounts for in vitro observations and cases of biochemical acantholysis, as well as the in vivo acantholysis in pemphigus induced by phenols. The mechanism involves the induction of interleukin-1a and tumor necrosis factor-a release by keratinocytes. These cytokines in turn have been shown to be involved in the regulation and synthesis of complement and proteases like plasminogen activator, which have been implicated in the pathogenesis of acantholysis in pemphigus vulgaris.