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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Suggest Medication For Recurring Pemphigus Vulgaris

i was diagnosed with pemphigus vulgaris a year ago & have been on predisone & cellcep for over a yr & still no relief. I think I have it under control but all of a sudden it flares up. its all in my mouth & throat.
can you help with any kind of diet or change in medication.
thank you
Mon, 16 Jun 2014
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Dermatologist 's  Response
Hello and welcome to healthcaremagic

I have gone through your query and I have understood it.

Pemphigus vulgaris is an autoimmune blistering disease characterised by flaccid intraepidermal bullae, which rupture easily and leave behind painful erosions. The erosions can involve the scalp, oral mucosa, lips, trunk and abdomen, groin and genitalia.

Oral steroids are the mainstay in the management of pemphigus. However, daily oral steroids have a lot of adverse effects like weight gain, weakening of the bones (osteoporosis), diabetes, high blood pressure, glaucoma, cataracts etc.

Immunosuppressants like cellcept(Azathioprine), Mycophenolate mofetil, cyclophosphamide, Methotrexate are steroid sparing agents, which are usually co-administered with oral steroids. Immunosuppressants decrease the requirement for oral steroids and allows the dose of oral steroids to be tapered Or ultimately stopped.

Alternative treatment modalities for Pemphigus are Dexamethasone Cyclophosphamide pulse therapy, Rituximab, IVIg etc.

Dexamethasone cyclophosphamide pulse therapy for pemphigus offers the only chance of long term cure/remission and is considered to be relatively safe and side effects are minor and few as compared to daily oral steroids.

Rituximab is a chimeric monoclonal antibody that targets CD20 molecule on B cell membranes resulting in cell death. Since Pemphigus is an antibody mediated disease, the use of Rituximab rituximab in patients with pemphigus is based on its ability to deplete CD20+ B cells that presumably produce pathogenic antibodies.

IVIg can rapidly control active Pemphigus by causing a selective and very rapid decline in the autoantibodies that mediate the disease by increasing the catabolism of pathogenic autoantibodies, responsible for pemphigus. This it provides an alternate and rapidly effective treatment for patients with active pemphigus unresponsive to conventional treatment.

IVIg is generally very safe. It should be more commonly utilized being a safe and effective therapy, however, the high cost of IVIg limits its widespread use.

Diet has no role whatsoever in the pathogenesis of pemphigus

Hope this helps

Regards
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Suggest Medication For Recurring Pemphigus Vulgaris

Hello and welcome to healthcaremagic I have gone through your query and I have understood it. Pemphigus vulgaris is an autoimmune blistering disease characterised by flaccid intraepidermal bullae, which rupture easily and leave behind painful erosions. The erosions can involve the scalp, oral mucosa, lips, trunk and abdomen, groin and genitalia. Oral steroids are the mainstay in the management of pemphigus. However, daily oral steroids have a lot of adverse effects like weight gain, weakening of the bones (osteoporosis), diabetes, high blood pressure, glaucoma, cataracts etc. Immunosuppressants like cellcept(Azathioprine), Mycophenolate mofetil, cyclophosphamide, Methotrexate are steroid sparing agents, which are usually co-administered with oral steroids. Immunosuppressants decrease the requirement for oral steroids and allows the dose of oral steroids to be tapered Or ultimately stopped. Alternative treatment modalities for Pemphigus are Dexamethasone Cyclophosphamide pulse therapy, Rituximab, IVIg etc. Dexamethasone cyclophosphamide pulse therapy for pemphigus offers the only chance of long term cure/remission and is considered to be relatively safe and side effects are minor and few as compared to daily oral steroids. Rituximab is a chimeric monoclonal antibody that targets CD20 molecule on B cell membranes resulting in cell death. Since Pemphigus is an antibody mediated disease, the use of Rituximab rituximab in patients with pemphigus is based on its ability to deplete CD20+ B cells that presumably produce pathogenic antibodies. IVIg can rapidly control active Pemphigus by causing a selective and very rapid decline in the autoantibodies that mediate the disease by increasing the catabolism of pathogenic autoantibodies, responsible for pemphigus. This it provides an alternate and rapidly effective treatment for patients with active pemphigus unresponsive to conventional treatment. IVIg is generally very safe. It should be more commonly utilized being a safe and effective therapy, however, the high cost of IVIg limits its widespread use. Diet has no role whatsoever in the pathogenesis of pemphigus Hope this helps Regards