Pemphigus Vulgaris: a rare disorder that caused a woman’s vulva to swell and blister

Pemphigus Vulgaris

Pemphigus Vulgaris is a disorder that causes woman vulva to swell and blister, but the sight of this redness and swelling on the vulva is not a welcomed one.


A recent case study published in BMJ Case Reports, tells the story of a woman who started noticing her vulva swelling, as well as yellow discharge and excruciating pain. When she consulted local urgent care and a general practitioner, she was misdiagnosed. One diagnosed her with folliculitis while the other diagnosed her with a yeast infection.


After receiving treatment from multiple doctors for six months, her symptoms did not improve. So she sought care at an emergency room (ER). At ER, she was attended by a dermatologist and a gynecologist. She was tested for STDs, bacterial and viral infections.


After many screenings came back negative, she was finally diagnosed with pemphigus Vulgaris, a rare autoimmune disorder that causes painful blistering on the genitals and other body parts.


Pemphigus Vulgaris

What is pemphigus Vulgaris?

Pemphigus is known as a collection of autoimmune disorders that cause mucous membranes (such as mouth, nose, eyes – all parts of the body that secrete mucous) and blistering of the skin, with pemphigus Vulgaris being the most common.


According to Anthony Fernandez, MD and a dermatologist at Cleveland Clinic, Pemphigus Vulgaris affects about four out of every million people in North America. This disorder particularly causes blistering that begins at the mouth and may gradually move to the skin and genitals.


According to the NORD (National Organization for Rare Disorder), blisters caused by pemphigus Vulgaris don’t itch, but painful and can make it difficult for anyone to chew and swallow things.


The GARD (Genetic and Rare Diseases Information Center) says pemphigus Vulgaris affects almost exclusively middle-aged and older adults. If you suffer from this disorder, what you will first notice is soft blisters that can appear on healthy skin or irritated skin.


Those blisters will be filled with fluid and when they rupture, you can experience a lot of pains and can be dangerous. It can cause your body to lose protein and fluids, and may potentially cause complication due to infection.


GARD also says, the overall cause of pemphigus Vulgaris is not yet known, but the cause of blistering of people with the disorder is known. Pemphigus Vulgaris makes the body treat proteins known as desmogleins as harmful foreign bodies. These desmogleins play a vital role in the body because they are the glue that holds skin cells together.


When there is an attack on desmogleins, skin cells separate and fluid will start collecting between the layers of skin, resulting in blisters that don’t heal, GARD says. Although rare, pemphigus Vulgaris flares can be caused by certain medications such as antibiotics and ACE inhibitor, which is a common blood pressure medication.


Age and genetic predisposition are few of the factors that increase one’s risk of having pemphigus Vulgaris. If you have an autoimmune disease, you are more likely to have pemphigus Vulgaris. Women are also likely to have it more than men.


Pemphigus Vulgaris

What causes pemphigus Vulgaris?

The immune system produces proteins known as antibodies, which normally attack harmful substances in the body including viruses and bacteria. Pemphigus Vulgaris occurs when the body immune system mistakenly makes antibodies against proteins in healthy mucous membranes and skin.


The antibodies break down bonds between the cells, and fluid collects between the layers of the skin, which leads to erosions and blisters on the skin. No one knows the real cause of the attack on the immune system.


Who is at risk for pemphigus Vulgaris?

Pemphigus Vulgaris is not contagious and cannot be transmitted from one person to another. Also, it does not appear to be transmitted from the mother to her child. But a person’s gene can put them at a higher risk of having this condition. This means you are more likely to have it if your parents or other members of the family had or have it.


This condition has no regard for ages, genders and races. However, it is more common in the following group:

  • Eastern European Jews
  • Middle-aged and other adults
  • People of Mediterranean descent
  • People who live in the rainforest in Brazil


Pemphigus Vulgaris treatment

Presently, there is no cure for pemphigus Vulgaris, but doctors are trying to reduce its symptoms and prevent complications through the available treatment methods. The treatment methods being employed include corticosteroids, which alleviate inflammation. Immunotherapy and immunosuppressive drugs, both of which tweak with the patient’s immune system, are also used by doctors to treat this disorder.


These drugs may have side effects including:

  • Cataracts
  • Osteoporosis
  • Glaucoma
  • An increased likelihood of becoming infected
  • Diabetes
  • Stomach ulcer
  • A loss of muscle mass
  • Water retention
  • Increased blood sugar

You may need to take supplements like vitamin D and calcium, eat a low-sugar diet, or take medications that can treat the side effects.


The good news is that, once the patient with pemphigus has been diagnosed and treated, he/she can resume a normal life. It means the disease can be controlled such that the patient will have nothing visible on the skin anymore. So if you are diagnosed with pemphigus Vulgaris, you have nothing to fear.


Pemphigus Vulgaris

Wound management

If your blisters are severe, the doctor may ask you to stay in the hospital to treat your wound. This treatment is like the treatment given to the patient with severe burns. You may need to receive electrolytes and IV fluids if you have lost too much fluid.


Some of the treatments of the blisters may include:

  • Pain medication
  • Soothing lotions
  • Soft-food diet
  • Wet dressings
  • Numbing lozenges for mouth blisters
  • Avoidance of too much sun exposure
  • Avoidance of spicy or acidic foods, which may irritate your blisters


If you have blisters in your mouth, which is keeping you from brushing or flossing your teeth, a doctor may recommend you for special oral treatment to prevent tooth decay and gum disease.


What are the complications of pemphigus Vulgaris?

The complications can be severe or fatal and may include:

  • Dehydration
  • Skin infections
  • Sepsis or the spread of infection through your bloodstream
  • Side effects of medication


The bottom line

If pemphigus is left untreated, it can become life-threatening. When there is a severe secondary infection, it can result in death. It is a lifelong condition because there is no cure for it yet. However, most people go into remission after receiving corticosteroids.


The condition usually improves within days of starting the corticosteroids. On average, the blisters stop forming in 2-3 weeks and the blister will heal slowly. The blister will heal within 6-8 weeks on average. But full healing may take up to a year or more and some people may need to stay on a low dose of medication for life.


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