Pemphigus Vulgaris

Pemphigus Vulgaris or PV happens when the binding of skin cells gets disrupted by antibodies produced by the immune system. The affected individual suffers from skin sores and blisters. But unlike other skin blistering problems, PV is a serious skin condition that is potentially deadly if left untreated. The disease requires thorough evaluation due to the similarities of its symptoms with other variations of Pemphigus.

Treatment for Pemphigus Vulgaris helps patients manage the symptoms and prevent the ailment from getting worse. It is aimed towards controlling the formation of skin blisters by means of medicines. The treatment regimen is closely supervised by a skin specialist to figure out what works best for the patient.

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Causes of Pemphigus Vulgaris

PV occurs when the immune system attacks itself. Under normal conditions, the immune system produces antibodies to fight invading bacteria and viruses and other harmful elements. With PV, the immune system produces antibodies that attack itself. In this regard, the antibodies assail a particular protein responsible for binding skin cells. This disruption causes the skin cells to break away and fluids will then start to fill the gap between the skin cells, hence forming blisters.

Experts do not know what triggers the immune system to do such.  They believe there are several influencing factors, which include:

  • Genetic predisposition
  • Other autoimmune disorders like rheumatoid arthritis and thyroid disorder
  • Old age, i.e. between 50 and 60; though it may also affect children
  • Certain medications like NSAIDs, pyrazolones, captopril and penicillamine

Effects of Pemphigus Vulgaris

PV causes unsightly and discomforting effects to those afflicted by it. The condition is manifested by:

  • Oral lesions

Around 50% to 70% of PV patients have oral lesions which are intensely painful. Some people get the oral lesions before the skin sores, though quite a few do not develop skin sores at all.

  • Skin sores

Skin sores are another symptom of PV and can be found on different parts of the body. Blisters are often found on the face, armpits, pressure joints, groins, buttocks and scalp. These skin lesions which could be several centimeters in diameter are not itchy. They are notably fragile and do not leave any scars once healed, however, they do lead to a pigmented skin color.

  • Blisters in mucous membranes

Blisters may also develop on the mucous membranes, such as those in the mouth, throat, in front of the eyes, anus and genitals. They could also appear on the larynx which causes voice hoarseness, as well as on the gullet, making swallowing extremely painful.

Suspecting individuals should visit the doctor immediately, especially if the condition is accompanied by fever, chills, malaise as well as muscle and joint aches. Early diagnosis and medical intervention could mean a big difference in preventing potential complications from happening.  These include severe dehydration, skin infections, medication side effects and sepsis.

Incidence of Pemphigus Vulgaris

Pemphigus Vulgaris is one of the varieties of Pemphigus, a group of autoimmune disease that causes skin blisters. Among them are benign familial pemphigus, paraneoplastic pemphigus, pemphigus foliaceous and pemphigus erythematosus. Of all the varieties of pemphigus, PV is the most serious and most common variety of pemphigus, accountable for nearly 70% of all the cases of pemphigus.

Still, PV is a relatively rare autoimmune disorder, affecting only about 1 to 5 in every 1 million people. Anyone may get PV regardless of race, but studies have shown that the condition predominantly occurs in the Jewish population and the Mediterranean regions.  It has been found to equally afflict males and females, specifically those between 50 and 60 years old. However, it tends to occur more frequently among female adolescents and rarely affects children.

Diagnosis of Pemphigus Vulgaris

Diagnosing PV is quite challenging as skin sores and oral lesions can arise due to a wide variety of reasons. PV is better diagnosed by a skin specialist who will conduct a series of tests to confirm the condition. The diagnosis procedure may involve antibody level blood test to measure the amount of PV auto-antibody present in the bloodstream. This test will help the doctor determine whether the individual has active or inactive PV. A skin biopsy may also be done to confirm that the blisters or lesions are due to PV.

PV is potentially deadly if taken for granted, most especially if it has lead to dehydration and serious skin infection. However, mortality is substantially reduced when PV is treated appropriately. Even so, patients need to talk with their doctor regarding their choice of treatment because some of the options have serious side effects.

Pemphigus Vulgaris treatment

Patients are presented with various treatment options which are all designed to reduce the associated symptoms and prevent complications from occurring. People with severe PV may be treated under a hospital’s intensive care unit or burn unit. The treatment is designed based on the specific needs of the patient.  The available treatment options include the use of:

  • Antifungal and antibiotics
  • Corticosteroids
  • Immunosuppresants
  • IV fluids/electrolytes
  • Plasmapheresis
  • Topical treatments for skin lesions

Pemphigus Vulgaris pictures

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