What is an autoimmune disease?
What is autoimmune disease? Autoimmune disease is caused by the immune system attacking the body's own organs or tissues. Autoimmune disease strikes one in every five Americans. The causes of autoimmune disease are unknown. When the immune system attacks the joints, it is called arthritis; when it attacks myelin (the insulation of the nerves) it is Multiple Sclerosis; when it attacks certain desmogleins (the “glue” that holds the skin together), it is a blistering disease called pemphigus.
How the immune system works (Illustrated)
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Essential information
about pemphigus
What you need to know
Pemphigus is a rare autoimmune bullous (blistering) disorder of the skin.
In simple terms, the body attacks the “glue” that holds its own skin cells together. This allows the cells to separate and form painful lesions (blisters) which will not heal. Pemphigus is not contagious, as is the case with all autoimmune disorders. Except for drug induced pemphigus, the cause is unknown.
The four main types of pemphigus are pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus.
Manifestations
Large areas of the skin and, in some cases, the mucous membranes (mouth and other moist linings of the body) may be affected. Lesions require the same care and treatment as severe burns. Before the availability of modern medicines most pemphigus patients died from infection.
The most common form of pemphigus is pemphigus vulgaris (vulgaris means common in Greek) which involves the mucous membranes.
Some patients may require hospitalization to deal with infection, the loss of body fluids or problems eating or breathing.
These disorders are chronic (ongoing) and the side effects of treatment can be severe, even disabling; but with proper treatment and a conscientious health care regimen, most patients regain control of their lives.
Diagnosis
Because pemphigus is rare and difficult to diagnose, it is often misdiagnosed. This can result in months of delay and incorrect treatment.
Accurate diagnosis is made by looking at several factors including,
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Clinical presentation (what the skin looks like)
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A skin biopsy (skin sample) which is examined under a microscope (to see if and where skin separation and damage is located)
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Immunofluorescence tests which show a specific pattern of staining
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A blood test for Autoantibodies against desmoglein 1 or 3.
Another indication of pemphigus, is when noninvolved skin next to a lesion separates easily when the skin is rubbed firmly with a finger or swab. This is called Nikolsky’s sign.
Frequency
Pemphigus affects males and females equally. The usual age when pemphigus is first noticed is between 30 and 60 years. The frequency is unknown but estimates vary from 10 to 30 patients per million per year worldwide.
The incidence is higher among certain ethnic groups including those of Mediterranean descent, Eastern European Asians, East Indians and especially Jews and Arabs.
The etiology (cause or origin) of pemphigus has been elusive. While there is a known genetic predisposition, pemphigus is very rare among genetic family members.
Seven drugs have been shown to causing or trigger pemphigus or pemphigoid. They are: Captopril Enalapril, Flupenthixol, Frusemide, Interleukin-2, Penicillins, Sulphasalazine and Penicillamine. For patients taking Penicillamine for more than 6 months, up to 7% will develop lesions which are identical to non-drug induced pemphigus.
Fogo Selvagem is an endemic (occurring in a local area) subtype of pemphigus foliaceus found mainly in remote areas of Brazil, Columbia, El Salvador, Paraguay, Peru and Tunisia.
Treatment
Pemphigus usually requires prompt and aggressive treatment; the extent of which depends on the severity of the disease. The goal of treatment is to heal the lesions, reduce symptoms quickly and prevent complications. The drug of choice is corticosteroids (steroids, for short). Prednisone is the most often prescribed steroid; brand names include: Deltasone, Orasone and Prednicen-M.
Prednisone is preferred unless there are life-threatening side effects.
An immunosuppressant, such as CellCept (mycophenolate mofetil) or Imuran (azathioprine), is often added.
Topical steroids or steroid injections into the lesions may also be used.
Steroids can cause severe physical and mental complications and should not be stopped suddenly. A balance must be found between the amount of medications required to control the disease and the desire to reduce the medications to reduce troublesome side effects, but stopping the formation of lesions is paramount.
The situation will vary from patient to patient and even within the same patient at different stages of the disease, and at different doses of medication.
Many patients require antidepressants to counter the mental side effects of steroids. Preventative measures and monitoring for depression should be started as soon as steroids are prescribed. See medications
Mouth ulcers may also necessitate intravenous feedings if severe and limiting oral intake. Antibiotics and antifungal medications may be appropriate to control or prevent infections.
Skin tests and tuberculosis tests may return false results due to steroids use. If contacted chickenpox and measles can sometimes cause severe complications. This is also true of the polio virus through a vaccination or by contact with someone who has recently taken the oral vaccine. Live vaccines should be avoided.
Diet
A diet of high protein, low carbohydrates, low salt and low fat is recommended. Patients are urged to eat foods containing high levels of calcium and potassium. Some patients are sensitive to garlic, onion and leeks (the Allium food group) which can cause or exacerbate itching or blister formation. Some patients report other foods as problematic.
Proper nutrition is important to provide the body with what it needs to repair damage and maintain optimum mental and physical health. Vitamin and calcium supplementation is usually a good idea.
Nontraditional treatment
Nontraditional medications and nontraditional treatments have not been shown to be effective against pemphigus. A delay or break in the traditional treatment protocol may result in a worsening of the patient's condition.
Nonprescription drugs and any herbs used should be reported to the doctor because some block or reduce the beneficial effects of medication. Any product which claims to “strengthen the immune system” should be avoided as the immune system is already overactive.
Pemphigus is sometimes confused with other autoimmune diseases of the skin, such as bullous pemphigoid, bullous lupus and Hailey-Hailey.
