Pemphigus: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

The condition known as pemphigus is characterized by blisters and ulcers on the skin or mucous membranes, such as those in the mouth or genitalia.

Pemphigus can affect anyone at any age, however it usually appears in those who are middle-aged or older. It is typically a chronic (long-lasting) illness, and if treatment is not received, it may even be lethal in some circumstances. Treatment with medication usually takes care of it.

Causes Of Pemphigus

Pemphigus is one kind of autoimmune illness. Normally, your immune system produces antibodies to protect you from harmful intruders like viruses and bacteria. But with pemphigus, the body produces antibodies that damage skin cells and mucosal membranes.

Pemphigus cannot be spread by others. The majority of the time, the illness's cause is unknown. Pemphigus can occasionally be brought on by penicillamine, angiotensin-converting enzyme inhibitors, and other drugs.

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Symptoms Of Pemphigus

When you have pemphigus, your skin and mucous membranes get blisters. The blisters often burst, leaving open wounds that could become infected. The following symptoms and indicators are present in two common types of pemphigus:

• Pemphigus vulgaris: This type typically begins with blisters on the lips and progresses to the skin or mucous membranes in the genitalia. The blisters hurt even though they don't generally itch. If you have blisters in your mouth or throat, it could be difficult for you to swallow and eat.
• Pemphigus Foliaceus: This type creates blisters all over the chest, back, and shoulders. Usually, blisters pain less than they itch. Pemphigus foliaceus does not induce mouth blisters.
• A blistering skin condition known as bullous pemphigoid, which primarily affects older persons, has the potential to be fatal and is not the same as pemphigus.

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Risk Factors Of Pemphigus

Pemphigus is more common in people who are middle-aged or older. Individuals of Middle Eastern or Jewish descent appear to be more susceptible to the sickness.

Diagnosis Of Pemphigus

Pemphigus is a rare condition that can be challenging to diagnose because it can coexist with several more prevalent conditions. Your doctor may recommend that you see a dermatologist or a specialist in skin conditions.

Your doctor will examine your mouth and skin, as well as talk to you about your medical history. Additionally, you may be required to take tests similar to these:

• Skin biopsy: For this test, a blister sample is removed, and it is examined under a microscope.
• Blood testing: One of the objectives of these tests is to find and identify antibodies in your blood that are known to be connected to pemphigus.
• Endoscopy: If you have pemphigus vulgaris, your doctor may advise an endoscopy to check for throat sores. This process entails placing a flexible tube (endoscope) down your throat.

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Treatment Of Pemphigus

Usually, the initial line of treatment consists of medications made to stop blisters from forming. Generally speaking, the sooner you begin, the greater the benefits. In cases where a medication was the source of your illness, stopping the medication's use may be enough to treat your pemphigus.

Medications

Using any or all of the following prescription medications depends on the type and severity of your pemphigus as well as any coexisting medical conditions:

• Steroid: For some people, corticosteroid cream may be adequate to treat mild episodes of the illness. For others, the mainstay of their treatment consists of oral corticosteroids such as prednisone tablets.
• Long-term or excessive use of corticosteroids can have serious negative effects, including diabetes, bone loss, increased infection risk, stomach ulcers, and a moon-face-like distribution of body fat.
• Immunosuppressants drugs: Your immune system from attacking healthy tissue can be stopped by taking medications such as azathioprine (Imuran, Azasan), mycophenolate (Cellcept), and cyclophosphamide. There may be severe side effects, like increased risk of infection.
• Other medications: Your doctor may suggest intravenous immunoglobulin, rituximab, or dapsone as a backup if first-line treatments aren't working for you.

Many individuals recover with treatment, however it might take years. For others, the medication needs to be taken continuously at a lower dosage to prevent the recurrence of the symptoms. Furthermore, certain individuals need hospital care, for example, to treat severe or infected sores.

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Complications Of Pemphigus

Pemphigus complications could include the following:

• Skin infection
• Sepsis, an illness spread through the bloodstream
• Malnutrition since eating is difficult because of painful mouth sores; unfavorable drug interactions, such as infection and high blood pressure
• If certain types of pemphigus are not treated, death

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