More information can be obtained from the IPPF and other sites.http://www.pemphigus.org/research/clinically-speaking/treatments/
There is no Pharmaceutical research focussed entirely on Pemphigus or Pemphigoid to our knowledge, although patients with the diseases can benefit from the advances in medical knowledge related to other auto-immune and other diseases and work goes on to identify the most successful drugs to treat blistering diseases. People respond to the medical treatments in different ways, so it is often a process of trial and error to find the regime which works best with the minimum amount of risk.
For mild cases, topical steroids are usually the first port of call. But for severe cases, the first treatment to get the blisters under control is a large dose of systemic corticosteroids, usually prednisolone. Large doses of steroids can result in emotional changes and other potential consequences, and their long term effects include gastric problems, bone problems and high blood pressure amongst other risk factors. Their use over a longer period is usually accompanied by other drugs to counteract the side effects.
The dose is then reduced slowly to minimise side effects. However, the prednisolone can seldom be stopped completely and most patients need a small maintenance dose to keep their disease under control. This dose varies from person to person, and depends partly on their weight.
It is dangerous to stop taking steroids suddenly, as your body may have stopped making its own corticosteroids and come to depend on the tablets you are taking for its daily requirement.
In order to minimise the dangers of corticosteroid use, other medications are often used as well. These are known as steroid-sparing drugs or adjuvant drugs and they work well when combined with corticosteroids.
They include the following:
- Immunosuppressives: azathioprine, cyclophosphamide, mycophenolate mofetil, ciclosporin and rituximab.
- Additional drugs: gold, methotrexate, tetracyclines (minocycline, or doxycycline) combined with nicotinamide, and dapsone.
Side effects. ALL these medications can have serious side effects, so patients must be monitored carefully by regular urine and blood tests, and blood pressure readings.
Plasmapheresis, intravenous immunoglobulin and rituximab may be considered if high doses of steroids are not effective. These may also be tried in combination with steroid tablets. Further research continues to find better treatments or combinations to treat Pemphigus and Pemphigoid.
A steroid cream may be used on skin blisters so that the dose of steroid tablets can be kept lower.
The use of nasal sprays or steroid mouthwashes containing antiseptic or local anaesthetic can also bring relief to the symptoms. Doctors will often also prescribe steroid creams for use on the skin. All of these must be used sparingly, and the patient can often be vulnerable to oral thrush when soluble steroids are used for rinsing the mouth or gargling.