All you need to know about Pemphigus.
Know your ailment well, so you can manage it better!!
Here we come with Pemphigus today!
What is Pempigus?
Pemphigus is a category of skin disorders that cause pus-filled bumps or blisters. Lesions typically grow on the skin, but in the mucous membranes (soft lining of the eyes, nose, mouth, throat, and genitals), they can also form.
The blisters are soft and quickly split open to form painful sores. They can spread across wide areas of the body without therapy and have a high risk of infection.
Pemphigus is an autoimmune disease that can occur in otherwise healthy individuals. Other autoimmune blistering skin disorders including bullous pemphigoid, lupus erythematosus, and Hailey-Hailey disease are also confused with it.
Pemphigus doesn’t get infectious. It’s a chronic condition that can be treated by continuing medical care.
What happens in the disease?
Pemphigus is an autoimmune (auto-means self) disease. In the upper layer of the skin (epidermis) and the mucous membranes, the immune system wrongly destroys cells. Pemphigus happens when:
- The immune system produces antibodies against desmoglein, a protein that normally forms the “glue” to keep skin cells attached.
- Skin cells separate from each other.
- Fluid may collect between the layers of skin, forming blisters that do not heal. In some cases, these blisters can cover a large area of skin.
In order to hold skin cells attached, the immune system develops antibodies against desmoglein, a protein that usually forms the’ glue.’ Separate the skin cells from each other.
Fluid can accumulate between skin layers, forming unhealable blisters. These blisters can cover a wide area of skin in certain instances.
Types of Pemphigus?
Doctors classify pemphigus into different types based on where and why blisters develop. In most cases, people only get one type of pemphigus.
Types of pemphigus include:
- Pemphigus Vulgaris: This type of pemphigus is the most common in the U.S. Blisters essentially always affect the mouth. Some affected people may also form blisters on the skin and in other mucous membranes. These lesions develop in deep layers of the skin. They can be painful and slow to heal.
- Pemphigus vegetans: This type of pemphigus is related to pemphigus Vulgaris. It involves lesions that are thicker and wart-like. These lesions usually form in the areas of the body with skin folds such as the groin and armpit.
- Drug-induced pemphigus: Medications cause blistering with this type of pemphigus. Some drugs that may cause this condition include penicillin and piroxicam. Blisters can develop up to six months after taking the offending medicine.
Pemphigus vulgaris in the mouth (above) and on the legs (below)
- Pemphigus erythematosus (Senear-Usher syndrome): This type of pemphigus involves blisters that develop on the upper back, chest, cheeks, and scalp. When lesions form, they are usually red and crusty.
- Pemphigus foliaceus: Blisters develop on the scalp and often the face, neck, and back. Lesions rarely appear in the mouth with pemphigus foliaceus. This type of pemphigus affects the outermost skin layer only. Small blisters may break easily to form crusted lesions that spread to cover large areas of skin.
Pemphigus foliaceus pictured above
- Endemic pemphigus (fogo selvagem): Endemic pemphigus is a form of pemphigus foliaceus that occurs more often in South and Central America, particularly Brazil. This form of the disease often affects multiple family members.
- Paraneoplastic pemphigus: This rarest type of pemphigus develops in people with cancer. Blisters in the mouth that resist treatment may be the first sign. If your doctors diagnose paraneoplastic pemphigus, they will look for signs of a tumor somewhere in your body. Removing the tumor often improves the symptoms of paraneoplastic pemphigus.
Who gets the disease?
Equally, Pemphigus affects both men and women. People of all races and ethnicities are also affected by the disease, however, the eastern European Jewish community and people of Mediterranean origin are more susceptible. In people who live in the rainforests of Brazil, a specific form of pemphigus also occurs more often.
In middle-aged and older adults, the disease usually begins to occur, but it can also be seen in young adults and infants.
What are the symptoms of Pemphigus?
Pemphigus causes blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.The signs and symptoms of two common types of pemphigus are as follows:
- Pemphigus vulgaris. This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful but don’t itch. Blisters in your mouth or throat may make it hard to swallow and eat.
- Pemphigus foliaceus. This type causes blisters on the chest, back and shoulders. The blisters tend to be more itchy than painful. Pemphigus foliaceus doesn’t cause mouth blisters.
Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects older adults and may cause death.
What causes Pemphigus?
Pemphigus describes an inflammatory disease. Your immune system usually develops anti corps to kill dangerous invaders, such as viruses and bacteria. But the body develops antibodies in the pemphigus that destroy the skin cells and mucous membranes.
Pemphigus is not infectious. In most cases what causes the disease is unclear.
Pemphigus is rarely caused by the use of inhibitors of the angiotensin-converting enzyme, penicillamine, and other medications.
What are the risk factors of Pemphigus?
Risk factors: Your risk of pemphigus increases if you’re middle-aged or older. The condition tends to be more common in people of Middle Eastern or Jewish descent.
What are the symptoms of Pemphigus?
Possible complications of pemphigus include:
- Infection of your skin
- Infection that spreads to your bloodstream (sepsis)
- Malnutrition, because painful mouth sores make it difficult to eat
- Medication side effects, such as high blood pressure and infection
- Death, if certain types of pemphigus are left untreated
How is Pemphigus Diagnosed?
For a variety of more common diseases, blisters occur, so it can be hard to diagnose pemphigus, which is rare. Your health care provider may refer you to a skin conditions specialist.
Your doctor will discuss your medical history with you, and will examine your skin and mouth. You will also undergo tests including:
- A skin biopsy. In this test, a piece of tissue from a blister is removed and examined under a microscope.
- Blood tests. One purpose of these tests is to detect and identify antibodies in your blood that are known to be present with pemphigus.
- An endoscopy. If you have pemphigus Vulgaris, your doctor may have you undergo endoscopy to check for sores in the throat. This procedure involves inserting a flexible tube (endoscope) down your throat.
What is the treatment for Pemphigus?
Treatment typically starts with medication designed to suppress the development of blisters. When it starts as early as possible it is usually more successful. If your condition was caused by the use of a drug, stopping its use could be enough to clear your pemphigus.
Medications
The following prescription medications may be used alone or in combination, depending on the type and severity of your pemphigus and whether you have other medical conditions:
- Corticosteroids. For people with mild disease, corticosteroid cream may be enough to control it. For others, the mainstay of treatment is an oral corticosteroid, such as prednisone pills.
Using corticosteroids for a long time or in high doses may cause serious side effects, including diabetes, bone loss, an increased risk of infection, stomach ulcers and a redistribution of body fat, leading to a round face (moon face). - Steroid-sparing immunosuppressant drugs. Medications such as azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide help keep your immune system from attacking healthy tissue. They may have serious side effects, including increased risk of infection.
- Other medications. If first-line drugs aren’t helping you, your doctor may suggest another drug, such as dapsone, intravenous immunoglobulin or rituximab (Rituxan).
How to cope up:
Blisters in the mouth can make your teeth uncomfortable by brushing and flossing so you should speak to your dentist about ways to keep your teeth and gums safe. You should avoid foods that could irritate blisters in your mouth.
Pemphigus and his treatments can weaken and cause missed work time, weight loss, sleep problems and emotional distress. Help groups will help you deal with the illness.
References:
https://www.niams.nih.gov/health-topics/pemphigus/
https://my.clevelandclinic.org/health/diseases/21130-pemphigus/diagnosis-and-tests
https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
https://rarediseases.org/rare-diseases/pemphigus/
By,
Gopala Krishna Varshith,
Content Developer & Editor,
Clipo.
