WHAT IS PEMPHIGUS: SYMPTOMS, CAUSES, DIAGNOSIS, AND MORE

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Pemphigus is an autoimmune illness that causes blisters and ulcers on the skin or mucous membranes, such as the mouth or the genitals.

Pemphigus can develop at any age, but it is most common in persons in their forties and fifties. It is usually a long-term (chronic) disorder, and some kinds can be fatal if not treated. Medication is usually effective in controlling it.


WHAT ARE THE TYPES OF PEMPHIGUS?

  • Pemphigus vegetans: This form is comparable to pemphigus vulgaris, but the lesions are thicker. These lesions typically appear in places of the body with skin folds, such as the groin and armpit.
  • Drug-induced pemphigus: Blisters can be caused by medications. Antibiotics and blood pressure medication are examples of substances that can cause this syndrome. Blisters might form months after taking the medication.
  • Pemphigus erythematosus (Senear-Usher syndrome): This type is a lupus overlap condition that causes blisters to form on your upper back, chest, cheeks, and scalp. Lesions are typically red and scaly as they form.
  • Pemphigus vulgaris: This is the most frequent type in the United States. Blisters always have an impact on your mouth. Blisters on the skin and other mucous membranes may occur in some people. These lesions form in the top layers of your skin. They can be painful and take a long time to heal.
Inside your mouth, Pemphigus vulgaris causes red and white fluid-filled blisters or open sores.

Blisters grow on your skin as a result of Pemphigus vulgaris. Blisters are most commonly found near your groin and on the skin on your legs.
  • Pemphigus foliaceus: This variety creates blisters on the scalp, face, neck, and back. Lesions in the mouth are uncommon. This type only affects your skin's outermost layer. Small blisters can readily rupture, becoming crusty lesions that spread to cover significant sections of skin.
Pemphigus foliaceus causes red to purple blisters to grow in clusters on your back, affecting the outer layer of your skin. These blisters expand quickly and cover a huge area of your skin.
  • Endemic pemphigus (fogo selvagem): This is a pemphigus foliaceus variant that is more common in South and Central America, particularly Brazil.
  • Paraneoplastic pemphigus: This is the most uncommon kind of pemphigus that occurs in cancer patients. In your mouth, severe blisters emerge. If your doctor diagnoses paraneoplastic pemphigus, they will look for evidence of cancer in your body.

WHAT ARE THE SYMPTOMS OF PEMPHIGUS?

The following symptoms may occur depending on the type of pemphigus you have:
  • Your skin has a fluid-filled lump or bubble (blister).
  • Blisters or lesions may leak clear fluid or faintly bleed.
  • Your skin around the blisters is thin and delicate, peeling in layers or scales.
  • Pink to red skin around the blister.
  • Sores with a crusty look
  • Pain on or near the damaged area of skin.
  • Skin itch.
Blisters and wounds are easily infected. Infection-related skin symptoms include:
  • White or yellow pus fills the blister and oozes out if it ruptures.
  • To the touch, there is pain or a burning sensation.
  • If the blister breaks open, a yellow crust forms on it.
  • The skin does not heal.
  • The blister grows or becomes larger in size.
Severe pemphigus symptoms include:
  • Fever.
  • Fatigue.
  • Muscle fatigue.
  • Light sensitivity and vision difficulties

WHAT ARE THE CAUSES OF PEMPHIGUS?

It is uncertain what causes pemphigus. According to research, genetics and environmental variables may have a role in your diagnosis.

Pemphigus is an autoimmune disease. This means that your immune system (antibodies) targets healthy cells in your body, mistaking them for foreign invaders. When your body assaults itself, you will develop pemphigus symptoms such as blisters or ulcers on your skin.

Certain drugs, such as penicillin, an antibiotic, piroxicam, a nonsteroidal anti-inflammatory drug used to treat rheumatoid arthritis, and blood pressure medications, can trigger the disease in rare situations.

According to certain research, certain HLA genes, which are genes that help form your immune system, predispose you to certain types of the illness.


WHAT ARE THE RISK FACTORS FOR PEMPHIGUS?

If you're in your forties or older, you're more likely to develop pemphigus. People of Middle Eastern or Jewish origin are more likely to have the illness.


WHAT ARE THE COMPLICATIONS OF PEMPHIGUS?

Pemphigus complications may include:
  • Skin infection
  • Bloodstream infection (sepsis)
  • Malnutrition, caused by severe mouth sores that make eating difficult.
  • Side effects of medications, such as high blood pressure and infection
  • If certain kinds of pemphigus are not treated, they might lead to death.

HOW IS PEMPHIGUS DIAGNOSED?

Blisters are associated with a variety of more frequent illnesses, making pemphigus, which is uncommon, difficult to identify. Your doctor may recommend you to a skin condition expert (dermatologist).

Your doctor will discuss your medical history with you and inspect your skin and mouth. You may also be subjected to tests such as:
  • A skin biopsy: A bit of blister tissue is extracted and examined under a microscope in this procedure.
  • Blood tests: One of the goals of these tests is to discover and identify antibodies in your blood that are associated with pemphigus.
  • An endoscopy: If you have pemphigus vulgaris, your doctor may recommend an endoscopy to check for throat sores. A flexible tube (endoscope) is inserted into your throat during this procedure.

WHAT ARE THE TREATMENTS FOR PEMPHIGUS?

Medication to reduce blister formation is usually the first step in treatment. It is often more successful if it starts as soon as feasible. If a medicine initiated your disease, discontinuing use may be enough to cure your pemphigus.

Medications

Depending on the kind and severity of your pemphigus and whether you have additional medical issues, the following prescription drugs may be used alone or in combination:
  • Corticosteroids: Corticosteroid cream may be sufficient to control moderate illness in some patients. For others, an oral corticosteroid, such as prednisone tablets, is the backbone of treatment.
Long-term or high-dose corticosteroid use can result in serious side effects such as diabetes, bone loss, an increased risk of infection, stomach ulcers, and a redistribution of body fat, resulting in a round face (moon face).
  • Steroid-sparing immunosuppressant drugs: Medications including azathioprine (Imuran, Azasan), mycophenolate (Cellcept), and cyclophosphamide help your immune system from attacking healthy tissue. They may have major adverse effects, such as increased infection risk.
  • Other medications: If first-line medications aren't working, your doctor may recommend a different medication, such as dapsone, intravenous immunoglobulin, or rituximab (Rituxan).
Many patients improve after receiving treatment, however it may take years. Others must take a lesser dose of medication indefinitely to keep their signs and symptoms at bay. And some people require hospital treatment, such as for severe or infected sores.


HOME REMEDIES FOR PEMPHIGUS

Here are some things you can do to help your skin and overall health:
  • Follow your doctor's wound care recommendations. Taking proper wound care can help prevent infection and scarring. Your doctor may offer recommendations for over-the-counter pain relievers.
  • Certain foods should be avoided. Spicy, heated, or abrasive meals may cause or aggravate blisters in your mouth.
  • Wash your skin gently. After washing with a gentle soap, use a moisturizer.
  • Take care of your skin. Avoid activities that may cause skin irritation.
  • Reduce your sun exposure. UV rays may cause new blisters to form.
  • Consult your dentist about how to maintain good dental health. Brushing your teeth may be tough if you have blisters in your mouth. Inquire with your dentist about what you can do to protect your dental health.

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