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11/26/2021 Chris Preston


Pemphigus - Symptoms, Causes, and Treatment

What is Pemphigus?

Pemphigus is a disease that causes blisters and sores on the skin or mucous membranes, like in the mouth or on the genitals.

Pemphigus could happen at any age, but it is most usually seen in people who are middle-aged or older. It tends to be a long-lasting (chronic) condition, and some types could be life-threatening without treatment. Treatment with medication generally controls it.



What is Pemphigus?


Pemphigus Symptoms

Pemphigus is responsible for blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which might ooze and become infected.

The signs and symptoms of two common types of pemphigus include:

  •    Pemphigus Vulgaris - This type generally starts with blisters in your mouth and then on your skin or genital mucous membranes. The blisters generally are painful but do not itch. Blisters in your mouth or throat might make it hard to swallow and eat.
  •    Pemphigus Foliaceus - This type is responsible for blisters on the chest, back and shoulders. The blisters have a tendency to become more itchy than painful. Pemphigus foliaceus does not cause mouth blisters.

Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects senior citizens and might cause death.

When should you see a doctor?

Consult your doctor if you have blisters inside your mouth or on your skin that do not heal.



Pemphigus


Pemphigus Causes

Pemphigus is an autoimmune disorder. Generally, your immune system produces antibodies to fight off harmful invaders, like viruses and bacteria. But in pemphigus, the body produces antibodies that affect cells of your skin and mucous membranes.

Pemphigus is not contagious. In most cases, it is unknown what triggers the disease.

Sometimes, pemphigus is triggered by the use of angiotensin-converting enzyme inhibitors, penicillamine, and other drugs.

Pemphigus Risk factors

Your risk of pemphigus rises if you are middle-aged or older. The condition tends to be more frequent in people of Middle Eastern or Jewish descent.



Autoimmune Disorder


Pemphigus Complications

Possible complications of pemphigus are as follows:

  •    Infection of your skin.
  •    Infection that spreads to your bloodstream (sepsis).
  •    Malnutrition, because painful mouth sores make it hard to eat.
  •    Medication side effects, like high blood pressure and infection.
  •    Death, if specific types of pemphigus are left untreated.

Pemphigus Diagnosis

Blisters happen with a number of more common conditions, so pemphigus, which is rare, could be difficult to diagnose. Your doctor might refer you to a specialist in skin conditions (dermatologist).

Your doctor will talk with you about your medical history and check your skin and mouth. In addition, you might undergo tests, including:

  •   A skin biopsy - In this test, a piece of tissue from a blister is removed and checked under a microscope.
  •   Blood tests - One objective of these tests is to discover and pinpoint antibodies in your blood that are known to be present with pemphigus.
  •   An endoscopy - If you have pemphigus Vulgaris, your doctor might have you undergo an endoscopy to check for sores in the throat. This procedure includes inserting a flexible tube (endoscope) down your throat.


Pemphigus Treatment


Pemphigus Treatment

Treatment generally starts with medications that are intended to suppress blister formation. It is generally more effective when it starts as early as possible. If the use of a drug triggered your condition, stopping use of it might be enough to clear up your pemphigus.

Medications

The following prescription medications might be used alone or in combination, depending upon the type and severity of your pemphigus and whether you have other medical conditions:

  •   Corticosteroids - For people with mild disease, corticosteroid cream might be enough to control it. For others, the mainstay of treatment is an oral corticosteroid, like prednisone pills.
    Using corticosteroids for a long time or in high doses might cause severe side effects, including 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 like azathioprine (Imuran, Azasan), mycophenolate (Cellcept), and cyclophosphamide help keep your immune system from attacking healthy tissue. They might have severe side effects, including an increased risk of infection.
  •   Other medications - If first-line drugs are not helping you, your doctor might suggest another drug, like dapsone, intravenous immunoglobulin, or rituximab (Rituxan).

Many people get better with treatment, although it might take years. Others need to take a lower dose of medication indefinitely to stop their signs and symptoms from returning. And some people require treatment in a hospital — for instance, to care for severe or infected sores.

If you or anyone you know is suffering from pemphigus, our expert providers at Specialty Care Clinics will take care of your health and help you recover.

Call 469-545-9983 to book a telehealth appointment for an at-home check-up.

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