Pemphigus vulgaris (PV) is an autoimmune disease, meaning the body’s immune system mistakenly attacks its own healthy cells. This condition causes painful blisters and sores on the skin and mucous membranes. PV is not contagious and cannot be spread from person to person.
Understanding Pemphigus Vulgaris
Pemphigus vulgaris is a rare autoimmune disorder where the immune system targets proteins called desmogleins, specifically desmoglein 3 (DSG3) and sometimes desmoglein 1 (DSG1). These proteins are found in the skin and mucous membranes, acting as the “glue” that holds skin cells together. Autoantibodies produced in PV disrupt these connections, leading to acantholysis, where skin cells separate and fluid accumulates, forming blisters.
Symptoms of PV include painful, thin-walled blisters, often filled with clear fluid. These blisters easily rupture, leaving raw, painful erosions or sores. While lesions can appear anywhere on the skin, they frequently develop inside the mouth, throat, nose, eyes, and genital areas. Mouth sores can be particularly painful, making eating and drinking difficult, and may precede skin blisters by several weeks or months.
Causes and Diagnosis
The exact cause of pemphigus vulgaris is not fully understood, but it involves a combination of genetic predisposition and environmental triggers. Certain genetic markers, particularly specific human leukocyte antigen (HLA) alleles, are associated with an increased risk. Environmental factors such as certain medications (e.g., penicillamine, ACE inhibitors), UV radiation, or some viral infections have been suggested as potential triggers. Stress and trauma might also play a role in triggering episodes.
Diagnosis of pemphigus vulgaris typically involves a combination of clinical examination and laboratory tests. A healthcare provider performs a physical exam and reviews symptoms, including examining painful blisters and erosions. A skin biopsy is a diagnostic step, where a small tissue sample from the edge of a blister or normal-appearing skin near a lesion is taken. This sample is examined under a microscope for characteristic features like acantholysis and the presence of autoantibodies through direct immunofluorescence (DIF). Blood tests, such as indirect immunofluorescence (IIF) and ELISA, also detect specific anti-desmoglein 1 and 3 autoantibodies circulating in the blood.
Treatment Approaches
Treatment for pemphigus vulgaris aims to suppress the immune system, reduce blister formation, and promote healing. The initial and often most effective treatment involves systemic corticosteroids, such as prednisone, which work quickly to control the disease. High doses may be used initially, with the dosage gradually reduced as the condition improves.
To minimize side effects from long-term corticosteroid use, immunosuppressants are prescribed alongside them. Commonly used immunosuppressants include azathioprine, mycophenolate mofetil, and rituximab. Rituximab, a biologic therapy, has shown significant effectiveness, particularly in cases that are difficult to treat with other methods. For severe cases, intravenous immunoglobulin (IVIG) therapy may be considered, administering pooled antibodies from healthy donors to modulate the immune response. Supportive care, including wound care for blisters and pain management, is also part of the treatment plan.
Clarifying Contagion
Pemphigus vulgaris is not contagious. It cannot be transmitted from one person to another through direct contact, touching blisters, or sharing personal items.
Unlike infections caused by bacteria, viruses, or fungi, pemphigus vulgaris is an autoimmune disorder. The blisters and sores seen in PV result from internal immune system dysfunction, not an external infectious agent. Therefore, there is no risk of spreading the disease through casual interaction or contact with affected skin.