Pemphigus skin disorder treatment 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.

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.

Emails
Q. I am trying to help a friend who's daughter was told she has Bechets disease and being treated for it. I am reading about it and came across pemphigus and pemphigoid . He is very concerned about her and is hoping she is getting the right treatment . I have read many of your past articles so your name is familiar. Google has brought me to you and I see pemphigus in one of your articles. I guess what I am asking is can you recommend a doctor who specializes in this or Bette's Disease.
   A. Sorry, I don't know of a doctor who is an expert on this topic.

My father has had been diagnosed with pemphigis for the past 6 years. He started with pemphigis v then pemphigis f. He is now in the hospital with a lung condition that the doctors feel is fungal. They have done tissue biopsies but they continue to come back benign for cancer. Is it possible that a person can develop pemhigis in the lungs? I have only read bits and pieces of information about para neo plastic pemphigis. Can this effect the lungs. His manifestations are a cavitation type lesion to the upper left lobe with an associated anemia. He lives in the upper peninsula of Michigan.
    This is not an area of medicine I have studied in enough detail to have a expert's opinion.