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What is pemphigus vulgaris and its treatment?

Q: I was diagnosed to have pemphigus vulgaris and treated with steroids. The lesions below the tongue and near the entry point of throat remain unhealed for nearly 20 months from the onset of disease. The lesions keep repeating in the mouth and near the gums. How to prevent this recurrence? Staying on 10-15 mg of steroids with 50 mg of cyclophosphamide for nearly another 3 years seems to be a must. Will it have any drastic side effects? I am however controlling sugar with acrabose and blood pressure with 12.5 mg of atenolol.

A:Pemphigus vulgaris is a rare autoimmune disease that is characterised by blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. It is an autoimmune blistering disease, which basically means that an individuals immune system starts reacting against his or her own tissue. Most patients first present with lesions on the mucous membranes such as the mouth and genitals. Several months later blisters on the skin may develop or in some cases mucosal lesions are the only manifestation of the disease. The most common mucosal area affected is the inside of the mouth but others include the eyes, food pipe, private parts and anus. Common features of oral mucosal lesions include:

  • 50-70% of patients get oral lesions blistering
  • superficial and often appears as erosions
  • widespread involvement in the mouth
  • painful and slow to heal
  • may spread to the larynx causing hoarseness when talking
  • may make it difficult to eat or drink I personally use a treatment modality known as Dexamethasone Cyclophosphamide Pulse therapy (DCP)where intravenous injections of DCP are given 3 days a month and during the rest of the days I use Cyclophosphamide (50 mg) daily. Of course you need to go to very competent dermatologist to take this treatment which I feel if given after taking due precautions and will have much lesser side- effects than daily steroid therapy that you are taking. Other medications that can be tried are:
  • Azathioprine
  • Dapsone
  • Tetracyclines
  • Nicotinamide
  • Plasmapheresis
  • Gold
  • Mycophenolic mofetil
  • Intravenous immunoglobulins

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