PEMPHIGUS - Autoimmune Disease
CANNABINOIDS - Symptom Relief
PEMPHIGUS - CANNABINOIDS FOR SYMPTOM RELIEF
Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.
Originally, the cause of this disease was unknown, and Pemphigus was used to refer to any blistering disease of the skin and mucosa. In 1964, a historic paper that changed the understanding of pemphigus was published. In 1971, an article investigating the autoimmune nature of this disease was published.
Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.
Pemphigus can occur at any age, but often strikes people in middle age or older. The most common form of pemphigus, pemphigus vulgaris tends to be more common in people of Middle Eastern or Jewish descent, though it can affect people of all races.
Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment, which may include medications or treatments similar to those used for severe burns. The less widespread your pemphigus is, the easier it may be to control.
If not treated, pemphigus can be fatal due to overwhelming infection of the sores. The most common treatment is the administration of oral steroids, especially prednisone, and often in high doses. The side effects of cortico-steroids may require the use of so-called steroid-sparing or adjuvant drugs. The immuno-suppressant CellCept (Mycophenolic acid) is among those being used.
Intravenous gamma globulin (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus. Mild cases sometimes respond to the application of topical steroids. Recently, Rituximab, an anti-CD20 antibody, was found to improve otherwise untreatable severe cases of Pemphigus vulgaris.
All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.
If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune suppressant drugs is sometimes used in an attempt to halt the rapid progression of bronchiolitis obliterans, including methylprednisolone, ciclosporin, azathioprine and thalidomide. Plasmapheresis may also be useful.
If skin lesions do become infected, antibiotics may be prescribed. Tetracycline antibiotics have a mildly beneficial effect on the disease, and are sometimes enough for Pemphigus Foliaceus. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.
Pain is a common part of the disease. Only one literature review on peer-reviewed articles reporting pemphigous pain management has been published in the professional medical literature. This can be read at: Pemphigus pain: a review on management.
- Pemphigus vulgaris. The most common form, pemphigus vulgaris usually begins with blisters in your mouth, which then erupt on your skin. Blisters can also break out on the mucous membranes of your genitals. The blisters typically are painful, but don't itch. Blisters in your mouth or throat may make it hard to swallow and to eat. Most common in people between ages of forty and sixty. More frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding.
- Pemphigus foliaceus. Least severe of the three types. This type doesn't usually affect mucous membranes. The blisters, which usually begin on your face and scalp and later erupt on your chest and back, usually aren't painful. They tend to be crusty and itchy. Desmogglein 1, the protein that is destroyed by the autoantibody, is only found in the top dry layer of the skin. It is less painful than PV. Often mis-diagnosed as dermatitis or eczema.
- Paraneoplastic pemphigus. This type is the least common form. This form, which is associated with certain types of cancer, causes painful sores on your mouth and lips and in your esophagus, as well as skin lesions. This form of pemphigus can also cause lesions in your lungs, resulting in progressive lung disease and making it difficult for you to breathe (dyspnea).
Exactly what triggers pemphigus is unknown, but it's an autoimmune disorder.
Normally, your immune system attacks foreign invaders, such as harmful viruses and bacteria. But in pemphigus, your immune system mistakenly produces antibodies that attack healthy cells in your skin and mucous membranes. In most cases, the antibodies attack proteins called desmogleins that bind skin cells to each other. The antibodies binding to the skin cause separation of the cells of the top layer of your skin (epidermis). This separation reaction is known as acantholysis.
Sometimes, pemphigus develops as a side effect of certain medications, such as blood pressure drugs or chelating agents. This type of pemphigus usually disappears when the medicine is stopped.
Paraneoplastic pemphigus is caused by additional antibodies to those seen in other forms of the disorder. It develops in people who have cancer, usually lymphoma or leukemia. In cases in which the cancer hasn't yet been discovered, the appearance of pemphigus blisters may alert doctors to look for a malignancy.
When to see a doctor
See your doctor if you develop blisters inside your mouth or on your skin. If you've already been diagnosed with pemphigus and are receiving treatment, see your doctor if you develop any of the following:
- New blisters or sores
- A rapid spread in the number of sores
- Achy muscles or joints
Widespread pemphigus may require you to stay in the hospital, where you may receive treatment similar to treatment for severe burns. The open sores make you highly vulnerable to infection, which, if it spreads to your bloodstream, may be fatal. Along with the medications listed above, you may be given:
- Fluids. Because you may have lost bodily fluids due to oozing of the sores, you may receive fluids through a vein (intravenously), as well as electrolytes — minerals such as sodium, potassium and calcium that maintain the balance of fluids in your body — and proteins.
- Intravenous feeding. This may be necessary if mouth sores make it too painful for you to eat.
- Anesthetic mouth lozenges. These can help control pain of mild to moderate mouth sores.
- Therapeutic plasmapheresis. In this process, the fluid part of your blood, called plasma, is removed from blood cells by a device known as a cell separator. The purpose is to get rid of the antibodies that are attacking your skin. The plasma is replaced with donated plasma or intravenous fluids.
For some people, treatment makes all of the blisters and sores disappear completely in a few months, and treatment can be stopped. Others have to stay on a lower dose of the medications indefinitely or their signs and symptoms will return
Treatments and drugs
Treatment, which aims at reducing signs and symptoms and preventing complications, is most effective when it begins as early as possible. The less widespread pemphigus is, the easier it may be to control. Specific treatment methods depend on the severity of the disease.?
If your pemphigus isn't too widespread, you may be able to remain at home for treatment. Medications may be used alone or in combination with each other to treat pemphigus. Commonly prescribed drugs include:
- Corticosteroids. The mainstay of treatment is usually corticosteroids, such as prednisone. However, using corticosteroids over an extended time or in high doses may cause serious side effects, including increased blood sugar, bone loss, an increased risk of infection, water retention, cataracts, glaucoma and a redistribution of body fat, leading to a round face (moon face).
- Immunosuppressants. These medications, such as azathioprine (Imuran), methotrexate (Rheumatrex) or mycophenolate mofetil (CellCept), help keep your immune system from attacking healthy tissue. These drugs may have serious side effects, including increased risk of infection.
- Antibiotics, antivirals and antifungal medications. These may be prescribed to control or prevent infections, particularly infection from staphylococcus bacteria or herpes viruses.
Pemphigus in domestic animals Pemphigus foliaceus skin eruption on the ventral abdomen of a dog
Pemphigus foliaceus has been recognized in pet dogs, cats and horses and is the most common autoimmune skin disease diagnosed in veterinary medicine. Pemphigus foliaceus in animals produces clusters of small vesicles that quickly evolve into pustules. Pustules may rupture, forming erosions or become crusted. Left untreated, pemphigus foliaceus in animals is life-threatening leading to loss of condition and secondary infection. Pemphigus vulgaris is a very rare disorder described in pet dogs and cats. Paraneoplastic pemphigus has been identified in pet dogs.
How Do Cannabinoids help?
Cannabinoids help with many of the symptoms.
- Cannabinoids are anti-inflammatories
- Cannabinoids are immunosuppressants.
- Cannabinoids are antifungal, antiviral, and antibiotic.
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