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PEMPHIGUS VULGARIS
About Your Diagnosis
Pemphigus vulgaris is a serious, bullous disease of the skin and mucous
membranes. It may be acute or chronic and is characterized by bullae (large
blisters) that burst. The lesions then become erosive and crusting. This disease
can be fatal unless the patient is treated with immunosuppressive agents (i.e.,
medications that reduce inflammation).
The cause is likely autoimmune (i.e., a condition in which your immune system
mistakenly attacks normal parts of the body, resulting in tissue injury or
disease). Certain medications can trigger the disease.
Pemphigus vulgaris is rare. It equally affects men and women, and typically
affects adults between 40 and 60 years of age. This condition is not infectious
or cancerous.
Diagnosis is usually based upon the appearance of skin lesions, testing of
skin specimens, and blood tests. Your doctor may perform a skin biopsy (i.e.,
removal of a small piece of skin or other tissue) for laboratory evaluation to
assist in diagnosis.
Medications that suppress your immune system have significantly improved
survival and recovery. Before immunosuppressive therapy, the disease was often
fatal.
Living With Your Diagnosis
Pemphigus vulgaris typically starts as painful erosions of the mucous lining
of the mouth, nose, throat, vagina, and anus. Many patients are unable to eat
normally because of the pain associated with chewing and swallowing. As a
result, patients can have weakness, fatigue, and weight loss caused by
nutritional deficits. Your skin may not be affected for several months. The skin
lesions are typically vesicles (i.e., small blisters) and bullae (i.e., large
blisters) that are flabby, easily broken, and weeping. The blisters may be
localized to certain areas of the skin for 6—2 months before becoming more
generalized. As the bullae rupture, they develop extensive erosions that weep
fluid, bleed easily, and crust. These erosions are prone to develop secondary
bacterial infections. Irritation of the mucous linings can cause symptoms such
as hoarseness, difficulty and/or painful swallowing, and bleeding.
Pemphigus vulgaris can be fatal if not aggressively treated with
immunosuppressive therapy. Because of the extensive erosions, patients are
highly susceptible to secondary bacterial infections, which may require
debridement (aggressive local cleansing of the wounds) and antibiotic therapy.
These infections are serious and have the potential to spread throughout the
body. Individuals may have nutritional deficits because of the pain associated
with eating and swallowing, resulting in weight loss, fatigue, and malnutrition.
Dehydration and abnormalities of electrolytes (such as sodium and potassium) can
occur as a result of nutritional deficits and extensive fluid loss from weeping
of the ruptured bullae. Scarring can also occur, although scarring is markedly
reduced if the bullae and erosions are allowed to heal spontaneously or to heal
with appropriate immunosuppressive therapy.
Treatment
Treatment consists of various immunosuppressive medications. These agents
lessen the effects of your immune system, reducing the formation of new
blisters, decreasing inflammation and pain associated with the blisters, and
promoting healing. In severe cases, patients require supportive measures
including intravenous fluids, aggressive debridement of the wounds, antibiotic
therapy to treat secondary bacterial infections, and intensive care of the
wounds. These measures often require hospitalization.
Medications lessen the effects of your immune system, thereby reducing
inflammation and suppressing the formation of new bullae. These agents are
effective and have significantly improved the survival and recoverability for
patients with pemphigus vulgaris. However, these medications may cause side
effects.
Your doctor may prescribe one or more of the following agents:
- Corticosteroids. High doses of these medications given as either tablet or
injection are effective in treating pemphigus vulgaris. Side effects are
more likely with higher doses, and include increased susceptibility to
infection, swelling, ulcers, diabetes mellitus, and osteoporosis (thinning
of your bones). Do not stop steroid medication without first consulting your
doctor, because abrupt cessation of these medicines can result in severe
fatigue, weakness, and low blood pressure. Topical steroid creams, lotions,
and ointments are used for milder cases.
- Immunosuppressive medications such as azathioprine, methotrexate, and
cyclophosphamide. These medications are potent suppressors of your immune
system and are effective in treating pemphigus vulgaris. When these agents
are combined with steroids, they reduce the total steroid dose required to
treat your condition, potentially decreasing side effects. Side effects of
these agents include increased susceptibility to infection and toxicity to
your body's organs, including the bone marrow (anemia and abnormally low
values of white blood cells and platelets), liver, and kidneys. Your doctor
will closely monitor your response to therapy and order laboratory tests to
check for possible toxic effects.
- Gold therapy for milder cases. These medicines are given as weekly
injections. Your doctor will restrict the total dose of gold to reduce your
risk of toxic effects to the liver and kidneys.
Your doctor may prescribe other medications to reduce symptoms and to treat
complications of pemphigus vulgaris. These medications include:
- Appropriate antibiotics to treat secondary bacterial infections.
- Medications to control pain and discomfort.
- Talc or powder to apply to the wounds and to bed linens. This can be used
in combination with topical antibiotic ointments to help treat secondary
bacterial infection.
No specific dietary measures can prevent or treat pemphigus vulgaris.
However, malnutrition and imbalances of fluid and electrolytes are common.
Therefore, your doctor will encourage you to eat and will closely monitor your
nutritional status. If signs of malnutrition develop, your doctor may recommend
hospitalization for more intensive fluid and nutritional care.
You will need to aggressively cleanse and monitor your wounds to prevent
complications such as infections or imbalances of fluids and electrolytes.
Your doctor may recommend one or more of the following measures:
- Cleansing baths.
- Debridement and local cleansing of skin wounds.
- Dressings including use of antibiotic ointments, topical steroids, and
talc.
- Correction of fluid and electrolyte imbalances.
- Plasmapharesis. In severe cases, plasmapheresis is used in conjunction
with medications. Plasmapheresis is a procedure performed by trained
personnel in which some of your blood is removed from your body. The blood
is then placed in a machine that can remove antibodies of your immune system
which are causing your pemphigus vulgaris. Blood is then reinjected into
your bloodstream. This treatment is effective in reducing the effects of
your immune system.
The DOs
- Take medications as prescribed by your doctor.
- Inform your doctor of all other medications, including over-the-counter
medicines, that you are taking. Continue these medications unless your
doctor instructs you to stop them.
- Read the labels of medicines and follow all instructions.
- Consult your doctor if you have any concerns, or if you have new or
unexplained symptoms that may be caused by side effects of the medication.
- Eat a well-balanced, nutritious diet. If lesions in your mouth or throat
are causing pain with eating or swallowing, follow a liquid or soft diet to
maintain nutrition.
- Keep follow-up appointments with your physician to evaluate your response
to therapy, to monitor for complications of pemphigus vulgaris, and to
assess for possible toxic effects of medications.
- Use general measures including cleansing baths, wound care, and dressings
as instructed.
- Closely check your skin and mucosal lesions for healing. Contact your
physician if you have any signs of secondary infection, including redness
around the lesions, purulent discharge (pus), increased pain or swelling of
the wounds or lymph nodes, and fever.
- Frequently clean towels, linens, and clothing to reduce your risk of
secondary bacterial infection.
The DON'Ts
- Do not stop your medication or change the prescribed dose without first
consulting your doctor. Do not exceed recommended doses of medications,
because higher doses can increase your risk of toxic effects.
- Do not abruptly stop steroids or immunosuppressive therapy, because you
may experience a rebound worsening of your condition. In particular, do not
suddenly stop steroids, because this action can have serious effects to your
health, including severe low blood pressure, fatigue, and weakness. Consult
your doctor before stopping these medications.
- Do not share towels, linens, or clothing with other individuals to reduce
the spread of infection.
- Avoid swimming or using a Jacuzzi until cleared by your physician.
- To decrease your risk of infection, avoid activities that may result in
contamination of your skin wounds and erosions.
- Do not drive or perform other potentially hazardous activities when taking
medication that can cause drowsiness or sedation (e.g., pain medications).
When to Call Your Doctor
- If you have any signs or symptoms of infection (see above).
- If you notice that lesions are becoming worse or if new lesions appear,
despite appropriate therapy.
- If you have signs and symptoms of systemic illness, including fever,
lethargy, confusion, or weakness.
- If you have new or unexplained symptoms, which may indicate a complication
of your condition or side effects from medications.
For More Information
Consult your primary care physician or your dermatologist to
learn more about pemphigus vulgaris. You may also contact: American Academy of
Dermatology
Attention: Communications Department
930 N. Meacham Road
Schaumberg, IL 60173
847-330-0230
Pemphigus vulgaris with oral erosions and no intact bullae. (Courtesy of
Department of Dermatology, University of North Carolina at Chapel Hill. From
Goldstein BG, Goldstein AO: Practical Dermatology, ed 1, St. Louis, Mosby—Year
Book, 1992. Used by permission.)
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