Generalized pustular psoriasis (GPP) is a rare and chronic (long-lasting) form of psoriasis that causes pus-filled bumps called pustules on your skin. The condition is also known as von Zumbusch psoriasis. It’s not contagious. GPP can also cause other symptoms that may become life-threatening if you don’t treat them right away.
How Is GPP Different From Other Forms of Psoriasis?
GPP is much less common than other forms of psoriasis but more serious.
With GPP, you may have breakouts of pus-filled bumps on your skin. These are called flares and they may come on quickly. You may also have inflammation and symptoms like fever, chills, nausea, muscle weakness, and joint pain.
Unlike other types of psoriasis, GPP can get worse quickly and lead to serious complications.
Complications include:
- A sudden drop in blood pressure
- Heart failure
- Hepatic failure
- Renal failure
- Respiratory failure
- Sepsis
In some cases, these complications may lead to death.
What Are the Common Symptoms of GPP?
The most common symptoms of GPP are pustules. They may be white or yellow. The skin around them may be swollen, tender, and discolored. The pustules can be painful.
You may have pustules on large areas of your body, which is common, or smaller spots on your fingers or your toes. You may also have bumps in your mouth and smooth, red patches on your tongue.
During a flare-up, the bumps may come together and open up, which create large clumps of pus. After they dry out, your skin may become red or purplish and form scabs. After that, your skin will shed, and the scabs will fall off.
GPP is different for everyone. You may have flares a few times a year or once every few years. Flares may last a few weeks, a few months, or longer. You may have pustules between flares or no bumps between flares.
Flares often come on suddenly. Your skin may feel fragile and sore. You may feel very sick.
Other symptoms of GPP include:
- Chills
- Dehydration
- Exhaustion
- Fever
- Headache
- Intense itching
- Joint pain
- Muscle weakness
- Nausea
- Rapid pulse
GPP can get worse quickly. You may need to go to the hospital, where a dermatologist will watch you until you get better.
If you have symptoms like fever, chills, serious itching, fatigue, muscle weakness, or a change in your heart rate, call your doctor right away.
What Triggers GPP?
Several things may trigger a GPP flare, but sometimes, they happen without any triggers.
Triggers of GPP include:
- Certain medications like methotrexate (Otrexup (PF), Rasuvo, Xatmep) and ustekinumab (Stelara)
- Corticosteroid treatment withdrawal
- Infections
- Injuries
- Low calcium levels
- Menstruation
- Pregnancy
- Skin irritants
- Smoking
- Stress
- UV light
- Vaccines
If you get GPP when you’re pregnant, it’s called pustular psoriasis of pregnancy. It usually happens in the third trimester.
Who Gets GPP?
Less than 3% of people with psoriasis have GPP.
It’s common to have plaque psoriasis and GPP. About 50% of people with GPP have both.
Arthritis, diabetes, and metabolic syndrome often happen in people with GPP.
Genes may play a role in who gets GPP. It’s been linked to mutations in the IL36RN gene, which is linked to skin inflammation.
GPP usually starts between ages 40 and 59 but can happen at any age. GPP is rare in children.
How Do You Treat GPP?
Doctors are still working on finding the best treatments for people with GPP. There isn’t a set of standard guidelines that everyone agrees on.
Your doctor will choose the best treatment for you based on your age, your overall health, and other medical conditions you have.
Your treatment plan may include short-term treatmen
t for GPP flares and long-term treatment to manage and prevent future flares.
Short-term treatment. Your doctor may give you medication to treat immediate flares. The treatment may relieve your symptoms, stop pustules from forming, and keep inflammation at bay. The goal is to control symptoms within a week of being treated.
Long-term treatment. Your doctor may give you medication to prevent new GPP flares and stop GPP from getting worse. They’ll monitor how you’re doing and watch for new flares so they can treat them quickly to prevent other problems.
What Medications Are Available for GPP?
Only one drug has been approved by the FDA to treat GPP. It’s a biologic drug called spesolimab (Spevigo). You can get spesolimab through an IV or at home by giving yourself injections.
Your doctor may give you other medications to help with your symptoms. They may recommend infliximab (Remicade), which is a medication that quickly reduces inflammation and bumps. Your doctor may recommend an oral retinoid, which is a pill that comes from vitamin A and helps get psoriasis under control.
Other medications include:
- Apremilast (Otezla)
- Biologics like ixekizumab (Taltz) or secukinumab (Cosentyx)
- Cyclosporine
Even though doctors often use topical medications to treat other types of psoriasis, they don’t use them to treat GPP. But if you have small areas on your skin that don’t respond to medication, your doctor may suggest putting a topical medication directly on the bumpy area.
Topical medications include:
- Corticosteroids
- Tacrolimus (Protopic)
- Vitamin D analogs
Your doctor may recommend home remedies to help with symptoms. They may include:
- Moisturizing creams
- Oatmeal baths
- Wet wraps
Another type of treatment is ultraviolet photochemotherapy, a type of light therapy, which can be done after your symptoms are stable. Light therapy may help clear up pustules. However, data is limited, and it’s unclear if light therapy is effective.
Why Is Getting Treated for GPP Important?
GPP flares can get worse quickly. It’s important to treat flares so they don’t develop into life-threatening complications like heart failure, respiratory failure, or sepsis.
During a flare, your doctor might recommend you go to the hospital so they can keep an eye on your vital signs and look out for signs of a serious infection or organ problems.
Show Sources
Photo Credit: iStock/Getty Images
SOURCES:
American Journal of Clinical Dermatology: “Treatment Options and Goals for Patients with Generalized Pustular Psoriasis.”
Dermatology and Therapy: “Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment.”
Journal of the American Academy of Dermatology: “Generalized pustular psoriasis: A consensus statement from the National Psoriasis Foundation.”
American Academy of Dermatology Association: “Pustular Psoriasis: Treatment.”
Cleveland Clinic: “Pustular Psoriasis.”
National Organization for Rare Disorders: “Generalized Pustular Psoriasis.”
National Psoriasis Foundation: “Generalized Pustular Psoriasis,” “Pustular Psoriasis.”
Psoriasis and Psoriatic Arthritis Alliance: “Pustular Psoriasis.”
