Pemphigus and Pemphigoid

Pemphigus and pemphigoid are rare conditions that occur when the body’s immune system attacks skin cells. There are many types of pemphigus and pemphigoid, including bullous pemphigoid, pemphigus vulgaris and others.

They all cause fluid-filled blisters, ulcers or rashes to form on the skin or mucous membranes (moist tissue found in the eyes, mouth, throat and other parts of the body).

Because they have similar symptoms, they’re often mistaken for one another. So it’s important to seek care from specialists — like Rush pemphigus and pemphigoid experts — who can tell them apart. An accurate diagnosis is key to finding treatments that work, and preventing complications such as chronic wounds, infection, scarring and vision problems.

Signs You Should Seek Care for Pemphigus or Pemphigoid

Pemphigus and pemphigoid have certain features that set them apart from other skin conditions. You should consult a dermatologist if:

• You develop recurring, unexplained blisters on any part of your body: Pemphigus and pemphigoid blisters can occur on the scalp, face, back, abdomen, arms and legs. They can also occur under the arms, on the genitals and in the mouth, throat or eyes.
• Your blisters are itchy or painful: Pemphigus blisters tend to be painful. They also break open easily, which can cause open sores on the skin. Pemphigoid blisters are often itchy and may occur with an eczema-like rash or hives. These blisters are firmer, meaning they don’t break open as easily, and can leave permanent marks on the skin. 
• Your blisters make it hard to get through the day: If blisters develop in your eyes, mouth or throat, you may have difficulty seeing, chewing or swallowing. Blisters on your hands or feet can make it hard to work, walk or take care of yourself. And blisters that break easily can leave painful, open sores on your skin.
• You’re at least 40 years old: Pemphigus usually affects people between 40-60 years of age, while pemphigoid tends to develop after age 60.

If your health insurance plan requires you to have a referral before seeing a dermatologist, start with your primary care provider. If you already have a referral, or you don’t need one, you can make an appointment with the dermatology team at RUSH.

Types of Pemphigus and Pemphigoid

Each type of pemphigus and pemphigoid affects different layers of the skin, and is triggered by different “autoantibodies” (special proteins that damage healthy cells). In other words, while these conditions might look similar on the body, they look very different under a microscope. 

Rush treats the full spectrum of these conditions, including:

• Pemphigus vulgaris
• Pemphigus foliaceus
• IgA pemphigus
• Paraneoplastic pemphigus
• Pemphigus vegetans
• Bullous pemphigoid
• Mucous membrane pemphigoid (also known as cicatricial pemphigoid)
• Gestational pemphigoid (also known as pemphigoid gestationis)
• Epidermolysis bullosa acquisita
• Anti-p200 pemphigoid

Because these conditions are rare and can be mistaken for one another — or for unrelated skin disorders — they’re often misdiagnosed. Fortunately, Rush dermatologists have special expertise in pemphigus and pemphigoid. We also offer up-to-date treatments that provide the best chance of getting these conditions under control.

Pemphigus and Pemphigoid Treatment at RUSH

RUSH offers every treatment available for pemphigus and pemphigoid, including new medicines that aren’t available at every doctor’s office. Our goal is to get rid of your blisters, relieve pain or itching, and prevent skin infection and other complications — while getting you off medications as soon as possible.

The Widest Range of Medications

Depending on the type of pemphigus or pemphigoid you have — and the severity of your symptoms — you may need one or more of the following prescription medicines:

• Topical steroids: Creams, gels or ointments applied to the skin can relieve pain or itchiness and help prevent infection. 
• Systemic steroids: Oral (taken by mouth) and injectable steroids can help reduce inflammation throughout the body. 
• Anti-inflammatory antibiotics: These medicines also help reduce inflammation throughout the body, but they have fewer side effects than steroids.
• Immunosuppressants: These drugs calm the immune system, which helps prevent new blisters from forming.
• Rituximab: This medicine belongs to a class of drugs called biologics; they help break the cycle of inflammation caused by a faulty immune system. Rituximab is usually used to treat adults with moderate or severe pemphigus. It’s given via IV infusion in a medical setting.
• Intravenous immunoglobulin: Also known as IVIG, this therapy is usually reserved for patients with severe symptoms who don’t respond to first-line treatments. Immunoglobulin is made from special cells in the blood that help fight germs. When given intravenously, it may help strengthen your immune system.

It may take several months for your pemphigus or pemphigoid treatments to work. Once your symptoms are under control, you and your provider will discuss whether you can stop taking some (or all) of your medications. 

Treatment Plans Tailored to You

When you choose Rush for pemphigus or pemphigoid care, you’ll receive customized treatments that follow the latest scientific guidelines — and fit your personal needs and goals. 

For example, research shows we should give certain patients a high dose of steroids right away, instead of several rounds of lower-dose steroids over time. This helps us get the disease under control (and taper you off steroids) more quickly. And it can prevent the side effects that may occur if you take steroids over a long period of time. 

We also work with you to make sure your treatments fit your lifestyle. For example, if your schedule makes it hard to have infusion treatments several times a month, we can find other medicines that are equally (or nearly as) effective. 

RUSH Excellence in Pemphigus and Pemphigoid Care

• Chicago’s only dermatology infusion center: RUSH s home to a unique, dermatologist-led infusion center that provides IV medicines solely to people with skin disorders. Because you won’t need to coordinate your treatments through a different infusion therapy expert (such as an oncologist or rheumatologist), you won’t need as many appointments. And our specialists aren’t just familiar with all of the IV therapies available for pemphigus and pemphigoid — they’re familiar with your medical history and treatment plan. We also work with your health insurance provider to ensure your infusions are approved ahead of time. 
• Research-driven care: Our dermatology team includes some of the country’s leading clinical and scientific experts on autoimmune blistering disease (a group of conditions that includes pemphigus and pemphigoid). Their research may pave the way for new, targeted treatments for pemphigus and pemphigoid. And their studies on autoimmune blistering disease have been published in more than 100 medical and scientific journals. 
• Convenient video visits: Our pemphigus and pemphigoid specialists make it easier for you to receive ongoing follow-up care by offering virtual (video) visits to eligible patients. This reduces the number of trips you’ll need to make to Chicago, and allows you to communicate with your provider from the comfort of home.