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Pemphigus: Symptoms, Causes & Treatment

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What is Pemphigus?

Pemphigus is a group of rare autoimmune disorders affecting the skin. An autoimmune disorder is a type of disorder or syndrome that causes the body’s immune system to attack healthy body cells as if they were invasive cells (x). In the case of pemphigus, the immune system attacks the skin and/or moist parts of the body, including the mouth, nose, throat and genitals.

This condition causes sores or blisters that can be itchy and very painful. The sores can be small and manageable or large, open and raw. In some cases, pemphigus presents a life-threatening situation (x).

Several forms of pemphigus have been identified. Most affect middle aged and older adults. There currently is no cure for the condition, but treatments are available to help keep it under control. In addition, some dietary supplements can help with managing the side effects of treatment.

Types of Pemphigus and Symptoms

Location and characteristics of the blisters determine the type of pemphigus (x). The catabolic state (breakdown of muscle proteins) that can result from this condition makes it potentially very dangerous to one’s health. In addition, secondary bacterial or viral infections can occur, leading to sepsis (x).

Pemphigus Vulgaris

Pemphigus vulgaris accounts for about 70 percent of all pemphigus cases and is the most common in North America. The symptoms of this type include (x, x):

  • Soft blisters that break easily
  • Often starts at the mouth and then spreads to other areas of the body including the scalp, chest, neck, back and genitals
  • Blisters are painful but not itchy
  • Skin peels easily when touched or pinched
  • Eating and drinking can be very painful
  • Stuffy nose that discharges bloody mucous
  • Hoarse voice due to sores in the throat

Pemphigus Foliaceus

Characteristics of this milder form of pemphigus include (x, x):

  • Blisters that start on the scalp and face before spreading to the skin on the chest and back
  • Does not cause blisters in the mouth or mucous membranes
  • Blisters that itch, but do not usually cause pain
  • Lesions that form crusts on the skin
  • Skin peels easily when touched or pinched

Fogo selvagem is a sub-type of pemphigus foliaceus, mostly found in rural areas of South America like Brazil. This type can affect children and younger adults and is thought to be triggered by environmental factors such as insect bites or medication (x).

Pemphigus erythematosus is another sub-type of pemphigus foliaceus and is also known as Senear-Usher syndrome. With this type, a butterfly-shaped rash often develops on the face. Some researchers describe it as having immunilogical features of both pemphigus and lupus (x).

Pemphigus Vegetans

The rarest type of pemphigus (accounting for 1-2 percent of cases), pemphigus vegetans (x, x):

  • Most often starts in the mouth before spreading to other areas of the body
  • Can leave thick plaques on the skin, especially under the arms and around the groin

Paraneoplastic Pemphigus

Also a rare type of pemphigus, this form is more commonly found in people with cancer or a benign (non-cancerous) tumor. It presents with (x, x):

  • Sores that develop on the mouth, lips, eyelids and/or esophagus
  • It can affect the lungs, causing a life-threatening situation
  • May be resolved partially or completely with removal of tumor
Symptoms of Pemphigus

Causes of Pemphigus

Pemphigus results from the body attacking its own healthy cells. Researchers still aren’t sure why this happens, but it appears to be a combination of genetic predisposition combined with exposure to something within a person’s internal or external environment (x).

Certain medications including penicillimine and ACE inhibitors may trigger the onset of pemphigus (x, x). In addition, such as in the case of paraneoplastic pemphigus, tumors are implicated in the onset of the disease.

Risk Factors

Pemphigus is not a contagious condition, which means that it cannot be transmitted from one person to another. Although the condition occurs worldwide, it is more common in specific groups (x) including:

  • People of Mediterranean descent
  • People living in rural communities in Central and South America
  • Middle aged or older adults

Pemphigus Diagnosis

The symptoms of pemphigus may resemble those of other skin diseases such as bullous pemphigoid or herpes (x). However, there are distinct differences between pemphigus and other skin disorders. For example, bullous pemphigoid also produces large, fluid-filled lesions, but the lesions do not open as easily as those of pemphigus. Herpes also has some symptoms that resemble those of pemphigus. However, herpes is not an autoimmune disorder (x).

Several tests can be performed to confirm pemphigus including (x):

Skin Examination

This involves using a cotton swab or finger to rub a patch of the skin that is not covered by blisters. If the skin peels off easily, then the patient may have pemphigus. However, a skin exam should be accompanied by additional tests.

Skin Biopsy

In this test, a doctor takes tissue samples from the blisters caused by what is suspected to be pemphigus for further analysis under a microscope.

Blood Tests

This involves checking the blood for desmogleins antibodies. The primary function of antibodies is to kill disease-causing pathogens. Patients with pemphigus have elevated levels of antibodies.

Endoscopy

Doctors perform an endoscopy by using a small, flexible tube to examine the inner lining of the mouth and throat.

Coping with Pemphigus

Pemphigus does not only have physical effects, but it can also have severe psychological effects, including distress, fear and loneliness (x). To cope with the disorder, find out as much as you can about it. There are many resources on the internet as well as libraries that provide valuable information about the condition. You may also talk to your doctor for tips on how you can join support groups and cope with the disease. Other recommendations for dealing with pemphigus include:

  • Avoid contact sports that could cause further damage to your skin
  • Avoid hot, spicy foods
  • Use a soft toothbrush
  • Talk to your doctor to prescribe painkillers if needed
  • Maintain good oral and skin hygiene
  • Apply talcum powder on your skin to prevent it from sticking to your clothes and bedsheets
  • Change and launder your towels, sheets and bed linens to prevent secondary re-infection
  • See a physician as soon as you suspect pemphigus
  • Attend support and pain management sessions

Pemphigus Treatment and Supplements

Although there is no cure, pemphigus can be managed through medication. Doctors can prescribe drugs to improve the symptoms of this condition (x).

Corticosteroids

This is one of the most effective treatments for pemphigus. It is usually available in pill form and can relieve the symptoms of the condition in just a few weeks.

Immunosuppressants

These drugs prevent your immune system from attacking healthy cells and tissues.

Biological Therapy

In this treatment, doctors inject a drug known as Rituxan to decrease antibody activities in the body.

Supplements for Pemphigus

No dietary supplement can prevent or treat pemphigus. However, certain vitamins and minerals can help the body counteract some of the side effects of medication.

Vitamin D

People with pemphigus often need to avoid exposure to direct sunlight since it can irritate their skin. Researchers also found that people with pemphigus generally have lower vitamin D levels to begin with, which could make the condition worse (x). As a result, supplemental vitamin D is recommended (x). A doctor can test a person’s current vitamin D levels to check if they’re within healthy range. Too much vitamin D is toxic and can lead to a variety of serious side effects.

To use vitamin D3 as a supplement, take 10 mg to 46 mg of this powder daily (amounting to a maximum of 5,000 IU), or as directed by a physician. Because of how concentrated this supplement is, it should be measured and consumed with care. Do not supplement this product if you do not possess an accurate milligram scale.

Calcium

The main medication to treat pemphigus, corticosteroids, can reduce bone density. This makes bone fractures and falls more likely, causing physical pain and a loss of independence. As a result, those on steroid medications are advised to make sure they consume enough calcium through food and/or supplements (x). Supplemental calcium comes in many forms such as calcium carbonate, calcium citrate and calcium aspartate. To learn which form is right for you and how to take it, more information can be found here.

Ginger

Another side effect of steroid medications is the development of stomach ulcers (x). Ginger is well known for its stomach soothing effects and can easily be incorporated into the diet, made into a tea or taken as a supplement. As a dietary supplement, take 1,000 milligrams of ginger root extract powder once daily, or as directed by a physician. To avoid any heartburn, take with at least 8 ounces of water.

The Bottom Line

Pemphigus is a potentially life-threatening autoimmune disorder. The exact cause is not known. This condition causes sores and blisters to develop on the skin, scalp and organs with mucous membranes such as mouth, nose and genitals. Researchers identified several different types and sub-types of this condition and are continuing to search for treatment options. Currently, there is no cure for pemphigus, but anti-inflammatory drugs and biologics can help control it. In addition, some dietary supplements like vitamin D and ginger are recommended to offset some of the treatment side effects.

About the author

Carey Ojuju


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