Pemphigus is a rare autoimmune disease that manifests by blisters and sores on the skin or mucous membranes, especially in the mouth or on the genitals, which are painful but do not itch. Then, the blisters can rupture easily, leaving open sores, which may ooze and become infected. Because of pain, patients will face difficulty eating and causing malnutrition. Thus, this disease tends to be a chronic condition if contracted with its complications, and worse it can be fatal if the infection spreads to the bloodstream.
Although it is rare, pemphigus is usually seen in people who are middle-aged or older. The cause of this disease is still unknown but there are triggers that can initiate the antibodies that normally fight off harmful invaders, go hay wired instead. There are few drugs that can induce pemphigus vulgaris, which are angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, cephalosporins and penicillamine. However, this is a very rare cause and this disease is more common in Jews and Middle Eastern due to some genetic factors. Cancer such as paraneoplastic pemphigus, infection or trauma also can trigger this disease.
Medicines for pemphigus Vulgaris are intended to suppress the immune system of the patient, hence suppress blister formation. First-line treatment is a high dose of systemic corticosteroid which can mimic the effect of the adrenal hormones your body naturally produces. Prednisone and Prednisolone are the most common systemic corticosteroid. Once most lesions have healed, the doctor needs to gradually reduce the dose to minimize the risk of side effects. Your doctor will monitor your clinical response and overall disease activity to determine the rate of dose reduction.
A common side effect of systemic corticosteroid is Type 2 Diabetes, which will diminish as the dosage of prednisone is reduced and will no longer be present when prednisone is discontinued. Thus, patients are advised to have a modification in their diet. Another commonly reported side effect of prednisone is weight gain, thus those who take prednisone need to create a high protein, low carbohydrate, low-fat diet, and a regular exercise program. Osteoporosis is also one of the common side effects of high dose steroids. taking supplements and a frequent follow up to di periodic bone density tests will prevent this side effect. Other than that, glaucoma and cataracts are also known side effects of prednisone.
Hence, the steroid dose needs to be optimised once pempheris vulgaris is controlled. Some patients need a small maintenance dose to keep the disease under control whereas some patients will go into remission. Other than reducing the dose, systemic corticosteroid also can be changed with topical steroids if only the mouth and nose are impacted or people with a mild disease such as steroid in the form of mouthwash, paste, ointment or aerosol.
Due to many side effects, steroid-sparing immunosuppressive drugs such as azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide can be an alternative to keep your immune system from attacking healthy tissue. They may have serious side effects, including increased risk of infection,or most common side effects are cough or hoarseness, fever or chills, lower back or side pain, missing menstrual periods, painful or difficult urination. If you use these drugs for a long time, you can experience blood in the urine, fast heartbeat, joint pain and unusual tiredness or weakness.
Other choices of the drug if first-line drugs are not helping you, your doctor can suggest dapsone, intravenous immunoglobulin or rituximab (Rituxan). Rituximab works as an immunosuppressant of B cells antibodies which are responsible for producing antibodies for the body. Hence, B cells that make antibodies in pemphigus will be destroyed. Intravenous Immunoglobulin (IVIG) therapy is a therapy to fight the hay wired antibodies, which is prepared from extracting the plasma from human blood. The treatment for pemphigus Vulgaris may take years but many people will get better with treatment, hence you need to always consult with the professionals.
