It is a rare autoimmune disorder characterized by blisters and erosions on the skin and mucous membranes. In addition to being the most common form of pemphigus vulgaris, it can have a significant impact on a person's quality of life. Below, you will find out more about it.
Immune cells mistakenly produce antibodies that target proteins called desmogleins, which are crucial for maintaining the integrity of skin and mucous membranes. As a result of this autoimmune response, cell-to-cell adhesion breaks down, causing blisters and erosions to form. It is unclear exactly what causes pemphigus vulgaris, but genetics and environmental factors may play a role.
As the name implies, pemphigus vulgaris is characterized by fluid-filled blisters that rupture easily, leaving painful erosions or ulcers behind. These blisters can appear on the skin, scalp, mouth, throat, genitals, or other mucous membranes. Initially, pemphigus vulgaris may appear as sores, which can make it difficult for a person to eat, drink, or speak. These blisters and erosions may recur over time and may be widespread.
Clinical evaluation, skin biopsy, and laboratory tests are usually needed to diagnose pemphigus vulgaris. In order to assess the characteristic appearance of blisters and erosions, dermatologists and other healthcare providers specializing in skin disorders will examine the affected areas of the skin and mucous membranes. A skin biopsy may be performed to obtain a sample of affected tissue for microscopic examination, which can help confirm the diagnosis and identify any underlying abnormalities. Pemphigus-specific antibodies can also be measured and disease activity assessed by blood tests.
Pemphigus vulgaris treatment involves suppressing the immune system, reducing inflammation, and promoting skin and mucous membrane healing. As a result, the immune system is suppressed and pemphigus antibodies are reduced by taking systemic immunosuppressive medications like corticosteroids. As an alternative to corticosteroids, azathioprine, mycophenolate mofetil, or rituximab may be used alone or in combination to control the disease. Supportive measures such as wound care, pain management, and dietary modifications may also be recommended.
The development of pemphigus vulgaris is primarily an autoimmune disorder, but genetic and environmental factors also contribute to its development, so preventing it is not always possible. However, avoiding known triggers such as certain medications, infections, or environmental factors that may exacerbate autoimmune activity can help reduce the risk of disease flare-ups in individuals with pemphigus vulgaris. Pemphigus vulgaris symptoms should be detected early and treated promptly to minimize disease severity.
