Generalized Pustular Psoriasis (GPP)

Generalized pustular psoriasis (GPP) causes scaly, bright red, thickened patches (plaques) and pus-filled bumps (pustules) on your skin. It can also cause inflammation inside your body. The affected areas can be very itchy and painful. These symptoms usually appear suddenly and can disappear quickly. You may also have muscle aches, a fever and feel really tired. Experts currently consider generalized pustular psoriasis (pronounced “PUHS-chuh-ler” “suh-RYE-uh-sis”) a type of psoriasis. But new research suggests it’s a unique and separate disease.

Generalized pustular psoriasis is rare but serious. According to the National Psoriasis Foundation, it affects about 1 in every 10,000 people in the U.S. Worldwide, experts estimate it affects between 1 and 7 out of every 1 million people.

If you have GPP, you need treatment at a hospital. You may even need round-the-clock monitoring in an intensive care unit (ICU). Without a proper diagnosis and treatment, it can cause life-threatening complications.

Another name for GPP is von Zumbusch psoriasis.

Symptoms of generalized pustular psoriasis

Generalized pustular psoriasis symptoms may include:

• A burning feeling in your skin
• Dry, tender, itchy and discolored skin, usually over large areas
• Skin plaques
• Pustules, which may develop on existing skin plaques or appear in new areas, and which may join together to create “lakes” of pus
• Fever
• Extreme tiredness (fatigue)
• Muscle weakness
• Joint pain

Your symptoms may suddenly go away and come back within a few days or weeks. But for many, this is a long-term (chronic) disease that comes and goes.

Generalized pustular psoriasis causes

GPP is a type of autoinflammatory disease. That means it happens because your immune system doesn’t act the way it should, and causes inflammation. Experts believe that genetic variations in several genes may cause it.

The most common genetic variation with GPP occurs in the IL36RN (interleukin 36 receptor antagonist) gene. IL36RN gives “instructions” for making cytokines. Cytokines are proteins that help manage inflammation. In healthy immune systems, inflammation happens when immune cells help protect your body from foreign invaders (like germs) or help heal damaged tissues. But a variation in the IL36RN gene may cause abnormal inflammation and trigger GPP symptoms.

What are GPP triggers?

Sometimes, GPP suddenly appears without any reason. But it may also have triggers, including:

• Infections
• Stress
• Corticosteroid (for example, prednisone) withdrawal
• Hormonal changes, including menstruation and pregnancy
• Sun exposure, including suntans or sunburns
• Smoking
• Low calcium levels

Risk factors

Anyone can have GPP. But it’s more likely to affect adults between 40 and 60. It’s also slightly more common in females.

About half of the people who have GPP may also have plaque psoriasis. It can also run in their biological families.

Complications

Without treatment, GPP may cause:

• Sudden drop in blood pressure (hypotension)
• Blood infection (septicemia)
• Low fluid volume (hypovolemia)
• Liver failure
• Kidney failure
• Congestive heart failure

These complications can be deadly.

If you’re pregnant and have GPP, risks may include:

• Congenital abnormalities (birth defects)
• Baby born before 37 weeks of pregnancy (preterm birth)
• The placenta not working as expected (placental insufficiency)
• Death before 28 days of life (neonatal death)

How doctors diagnose GPP

A healthcare provider will:

• Review your health history, including any new or changes in medications
• Ask about any symptoms, including if you’ve had flares before
• Ask if any blood relatives have a history of plaque psoriasis or psoriatic arthritis
• Perform a physical exam, which includes checking your vital signs

If they suspect GPP, they may recommend blood tests and a skin biopsy.

Blood tests can help your provider discover abnormal:

• Fluid levels
• White blood cell levels
• Electrolyte levels
• Liver enzymes

If the labs are abnormal, they may be signs of serious GPP complications.

A skin biopsy can help confirm the diagnosis. It rules out other conditions that cause pustules.

How is GPP treated?

A healthcare provider will first check your overall health and vital signs. If there are any complications, you may first need:

• Medications to treat a fever and pain
• Antibiotics to treat a bacterial infection
• IV fluids to balance your fluids and electrolytes

Next, your provider will likely start treatment that specifically targets GPP:

• Oral medications, such as acitretin, cyclosporine and methotrexate
• Biologics (injectable antibodies), including TNF inhibitors and IL-36R inhibitor

Your provider may also apply creams, lotions or gels to help calm your affected areas. These may include:

• Corticosteroids (for example, hydrocortisone)
• Vitamin D
• Tacrolimus

Recovery time

GPP flares can last from weeks to months. Once the symptoms go away, it’s in remission. But it’s not gone. You could have another flare in the future. It’s not easy to predict how long you’ll go between flares. Your remission timeline may last a few months to years.

When should I see my healthcare provider?

Call your provider if you notice any skin changes, especially a rash that doesn’t go away or gets worse. Get help right away if you have a skin rash and signs of serious complications, including:

• Fever and chills
• Shortness of breath
• Chest pain
• Heart palpitations
• Abdominal pain
• Nausea and vomiting
• Peeing less than usual or not at all
• Yellow tint to the whites of your eyes and skin (jaundice)
• Mental changes, including trouble concentrating or confusion
• Swelling, especially around your hands or ankles

It’s also a good idea to see your provider if they diagnose you with GPP and you notice possible triggers. They can confirm any triggers and help you avoid them.

What can I expect if I have generalized pustular psoriasis?

There’s no cure for GPP. Symptoms may go away and come back throughout your life. But healthcare providers are still learning about it. This includes new treatment options that can help ease your symptoms and reduce your risk of complications.