Pemphigus skin disorder treatment
June 20 2017 by
Ray Sahelian, M.D.

 

Pemphigus vulgaris is a life-threatening autoimmune blistering skin disease. It 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.

 

Scientifica (Cairo). 2013. Bullous skin diseases: classical types of autoimmune diseases. The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemi)desmosomes. Koch's postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch's postulates are fulfilled, and, therefore, these bullous skin diseases are to be considered classical autoimmune diseases within the wide and expanding spectrum of autoimmune diseases.

 

Treatment of Pemphigus vulgaris skin disorder
The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents.

 

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.

 

Clinical, Cosmetic and Investigational Dermatology 2015. Management of pemphigus vulgaris: challenges and solutions. Systemic corticosteroids remain the gold standard treatment for pemphigus vulgaris. Azathioprine and mycophenolate mofetil are the first line of steroid-sparing treatment. Rituximab is extremely effective in recalcitrant pemphigus, when other treatments fail to control the disease. The European Dermatology Forum recommends tapering prednisolone by 25% every 2 weeks after the consolidation phase, and a 5 mg reduction every 4 weeks when the dose is reduced to <20 mg. If the patient relapses, options include increasing steroids back to the previous dose, adding an immunosuppressant if using steroid monotherapy, or replacing a first-line immunosuppressant by another if already on combination therapy.

 

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.

 

I am writing you with high hope that I will get a help in recommending a natural treatment for my mother who was diagnosed few months back with pemphigus. After taking corticosteroids (prednisone) for few months, she is reducing the dose...now she is taking 40 mg every second day. At the beginning she started with 60 mg .the blister started to appear again. I am afraid that corticosteroids might destroy her digestive system. Is there any alternative treatment for this disease?
   As of 2017 I am not aware of a natural solution to this skin disease.

 

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.

 

I'm a 37 yr old male living in Arizona and I have been diagnosed with pemphigus foliaceus. I currently take cellcept 2000 mg in the morning and 2000 mg at night, and I take vitamin c, d3, fish oil, and lysine. Do you know off any other supplements to take that might help or more importantly is there any food, drinks, or environmental things I should stay away from. I go to a dermatologist at the U of Arizona but they seem to not know much about pemphigus or any triggers.
   A. I am not aware of an effective natural treatment at this time.