What is pemphigus?
Living with a blistering autoimmune skin disease like pemphigus can be difficult. The fragile blisters it causes break easily and leave painful sores. These can appear on the skin or in moist areas like the eyes, nose, mouth, and throat, making eating, drinking, and speaking painful. If raw, oozing sores become infected, they can lead to serious complications. This long-term condition can take a toll on your daily life, but with the right care plan, pemphigus can be managed.
Dermatologists who specialize in autoimmune skin disorders can diagnose and treat pemphigus. Because the disease can affect the mouth and eyes, care may be coordinated with dentists or ophthalmologists. Penn Dermatology specialists treat all types of autoimmune blistering diseases, including the rarest and most challenging forms.
Pemphigus symptoms
Different types of pemphigus produce different symptoms, which can include:
- Fluid-filled bumps or blisters
- Oozing or crusting of open sores
- Peeling or shedding skin
- Itchy skin
- Painful sores or skin
- Darkened spots or patches
- Infected sores
- Sepsis if the infection spreads to the bloodstream
Types of pemphigus
There are several types of pemphigus, based on where the blisters form and which layer of skin they affect.
Pemphigus vulgaris is the most common type in the U.S. It usually begins with fluid-filled blisters and painful sores in the mouth. Blisters may then appear on the skin.
Pemphigus foliaceus affects only the top layer of the skin (subcorneal). It causes painful or itchy blisters on the head, trunk, and extremities. Sores rarely appear inside the mouth or other moist areas.
IgA pemphigus is uncommon. It causes painful, itchy blisters that often appear on the torso and nearby limbs. Red patches or ring-shaped patterns may also develop.
This rare form is usually linked to an underlying tumor or cancer like lymphoma. Paraneoplastic pemphigus causes blisters on the skin, in mucous membranes, and in some internal organs, like the lungs (bronchiolitis obliterans).
Certain medications, like thiol-containing drugs, can trigger this form of pemphigus. Blisters may appear weeks or months after starting the drug. Drug-induced pemphigus is often less severe and may have a better outcome than other forms.
What causes pemphigus?
Pemphigus happens when the immune system mistakenly attacks the proteins that help skin and mucous membrane cells stick together, causing blisters to form. Why the body attacks itself is unknown, but research suggests both genetic and environmental factors may play a role.
Diagnosing pemphigus
If your care provider suspects pemphigus, they may gently rub the skin near a blister to see if it peels, a sign that helps support the diagnosis. They may also take a small tissue sample from a blister and nearby healthy skin to examine under a microscope. In some cases, a blood test may be ordered to look for antibodies linked to pemphigus. Because pemphigus is rare, they may also check for other possible conditions before confirming the diagnosis.
Treatment for pemphigus
Pemphigus is usually treated with medication to help stop new blisters from forming and to heal the ones you already have. This often includes steroids and drugs that calm down the immune system. If a drug caused your pemphigus, stopping it might help your skin heal. In some cases, wound care, antibiotics, or other supportive treatments are needed if the skin becomes infected.
Specialists in blistering autoimmune disease care
When you’re facing a rare and potentially serious autoimmune disease like pemphigus, it’s important to choose a provider with experience. At Penn Dermatology, we specialize in diagnosing and treating all types of autoimmune blistering diseases.
Physicians from across the southeastern Pennsylvania and New Jersey regions and beyond refer complex cases to us. Several of our dermatologists serve on boards or participate in committees of the International Pemphigus and Pemphigoid Foundation.
Choosing Penn Dermatology means you’ll receive expert, compassionate care. We are one of the few dermatology departments in the nation with dedicated research labs and in-house dermatopathology services. Among the best in the country, our dermatopathologists use advanced techniques, including direct and indirect immunofluorescence, to analyze skin biopsies and accurately diagnose pemphigus.