Generalized pustular psoriasis (GPP) is the rarest form of psoriasis, a chronic autoimmune skin condition. Unlike other forms, GPP can be severe or even life-threatening.
The disease causes flares of widespread inflammation of the skin, leading to red patches and pustules on large parts of your body. That inflammation usually comes with other symptoms. Sometimes, those symptoms only affect your skin, causing itching and a burning sensation. Other times, the disease can affect your whole body, bringing with it fever, fatigue, muscle weakness, and more. Severe flares can even lead to kidney, heart, or respiratory failure.
Who gets GPP?
GPP affects about 1 in 10,000 people in the U.S. While it can develop at any age, you're most likely to get it between the ages of 40 and 59. People assigned female at birth (AFAB) have a slightly higher risk of getting the disease than people assigned male at birth (AMAB).
What Are General Pustular Psoriasis Types?
There's no widely agreed upon classification of the different types of GPP, which include:
Von Zumbusch, or acute GPP
This is the most common type of GPP and the most dangerous, as it can lead to life-threatening complications such as sepsis (from an infection) or a sepsis-like syndrome. It comes in acute flares followed by periods in which the disease causes few or no symptoms. Symptoms can include:
- Fatigue
- Fever
- Chills
- Loss of appetite
- Nausea
It can also cause changes in your eye, such as conjunctivitis, iritis, and uveitis (inflammation of the outer surface of the eye, the iris, or the middle layer of the eye).
Annular GPP
This type of GPP is chronic and relapsing, which means it doesn't go away completely and symptoms can come back. It's more common in children than adults, usually arising between the ages of 6 weeks and 10 years, with the average around 6 years. Annular GPP causes symptoms on your skin, most often on your belly area, trunk, and lower arms and legs. They look like ring-shaped (annular) plaques, which are thick, raised areas of skin surrounded by red, scaly skin or pustules.
The symptoms of annular GPP are generally less severe, but in some cases it has developed into acute GPP. It usually responds well to treatment. Annular GPP is also called Lapière-Millian.
Chronic acral GPP
With this type of GPP, the pustules appear first on your hands and feet, and then spread over time to other areas of the body to a generalized pustular flare.
Mixed-pattern GPP
This type can start as one pattern, like annular, but then develop into another type of acute flare-up.
Pustular psoriasis of pregnancy
Also known as impetigo herpetiformis, this type usually occurs in the third trimester of pregnancy. It's possible to get it as early as the first month. This type of GPP often lasts until your child is born, though it may continue after childbirth.
Like von Zumbusch GPP, this type of GPP can be deadly. In fact, some experts say that this is the same disease as von Zumbusch. If you develop it during pregnancy, you're likely to get it again in future pregnancies. Oral contraceptives also may trigger it.
What Are the Causes of Generalized Pustular Psoriasis?
Experts don't yet know for sure why GPP happens, but several things likely play a part, either in the development of the disease or as triggers that lead to your flares.
Your genes and your immune system
You have a gene called IL36RN, which normally keeps inflammation under control. If you have a faulty version of this gene, you will be more likely to develop GPP. It often starts in childhood. If you have GPP symptoms in your mouth and you don't have plaque psoriasis, you may have a mutation in your IL36RN gene.
Another gene scientists think may play a role in GPP is called CARD14. It's also involved in your immune system and in managing inflammation. Mutations in this gene have been tied to GPP in both adults and children. Research has also linked problems with other genes to GPP. These genes include:
- AP1S3
- MPO
- SERPINA3
Your environment
Events and features of your environment can act as triggers of GPP. Some examples include:
- Smoking
- UV radiation from the sun
- Sunburns
- Emotional stress
Medications
A number of medications have been linked to GPP. One of the most commonly known medication-related triggers is stopping corticosteroid medications, including oral steroids or strong topical corticosteroids. These medicines suppress your immune system and are typically prescribed for short periods to control inflammation. Other immunosuppressant drugs that may trigger GPP when stopped include:
- Cyclosporine (Gengraf)
- Methotrexate, which is usually prescribed for rheumatoid arthritis, psoriasis and some cancers. It can trigger GPP in rare cases.
- Rituximab (Rituxan)
- Ustekinumab (Stelara), which can trigger GPP in rare cases
- Medications called tumor necrosis factor alpha (TNF alpha) inhibitors and interleukin-17 (IL-17) inhibitors, prescribed for plaque psoriasis, rheumatoid arthritis, and Crohn's disease
Other types of medicines, hormones, or other substances linked to GPP include:
- Antibiotics, including amoxicillin, ceftriaxone, and oxacillin
- Aspirin
- Codeine, a pain medication
- Hydroxychloroquine
- Lithium
- Progesterone
- Salicylates, a naturally occurring organic acid that is found in fruits and vegetables like blueberries, mushrooms, and avocados
- Terbinafine, an antifungal medication
Infection
The following infections have been linked to GPP:
- Cytomegalovirus (a type of herpes)
- Chickenpox and shingles (varicella zoster virus)
- Strep throat (streptococci infection)
- Mono (mononucleosis, caused by the Epstein-Barr virus)
- COVID-19 (SARS-CoV-2)
What Are the Symptoms of Generalized Pustular Psoriasis?
The symptoms of GPP occur or worsen when your disease flares up. This usually happens suddenly.
GPP symptoms can vary, but you can expect the disease to cause large areas of reddened skin covered with tiny, painful, white bumps filled with sterile pus. These pustules also can form in your mouth, while reddish, smooth patches may develop on your tongue.
The symptoms of GPP usually follow this course:
- A burning sensation develops on your skin, which becomes tender and dry.
- A high fever starts, along with a general feeling of being unwell.
- Areas of skin previously affected by the disease become covered with pustules.
- Redness and inflammation of your skin gets worse.
- Pustules begin to cover previously healthy skin, particularly in skin folds and around your genitals.
- Pustules may appear as individual bumps, or they may merge to form what's called "lakes of pus."
- Pustules may form within circular skin lesions, on areas of reddish raised skin called plaques, or as part of widespread redness on your skin.
- Later, the pustules usually will dry up, forming scales and redness before they fall off.
While the above symptoms mostly involve your skin, GPP causes other symptoms, including:
- Thickened nails, with pus underneath
- Red, map-like patches or bumps on the tongue's surface (geographic tongue)
- Fever
- Fatigue
- Headache
- Chills
- Nausea
- Diarrhea
- Pain in your joints
- Weakening of your muscles
- Swelling (edema) of your legs if heart failure develops
- Inflammation of your eyes
- Faster-than-normal pulse
- Low blood pressure
- Yellowing of the skin and the whites of the eyes (jaundice)
- Intense itching
How long do symptoms last?
Once a flare begins, skin symptoms take about a week to fully spread. Flares usually last a few weeks but can continue for months before the disease settles down and goes into remission. In some cases, the pustules that form during a flare will remain on your skin between flares.
What Triggers General Pustular Psoriasis Flares?
GPP itself doesn't have a trigger to start, but some people do have triggers that will cause flares. Not everyone with GPP has clear triggers. You may or may not know what your triggers are.
How Is Generalized Pustular Psoriasis Diagnosed?
Diagnosing GPP can be difficult. For one thing, the disease is rare, so your doctor may not expect it. It also causes a great number of symptoms that can vary from person to person, making it tough to identify and easy to confuse with other conditions.
When you see your doctor, you will discuss your symptoms; what was going on when your symptoms began, such as the types of medications you were or are taking; and your medical history. For example, if you have plaque psoriasis, that can be a clue that GPP may explain your symptoms.
Next, your doctor will take a close look at your skin and the pustules, and do a physical exam. They may also order several tests. While there's no blood test to diagnose GPP, blood tests can help to confirm your doctor's suspicions that you have GPP and rule out other causes. Blood tests also help evaluate how the disease is affecting you.
These tests could include checks of:
- Complete blood count (CBC)
- Erythrocyte sedimentation rate, a measure of inflammation
- C-reactive protein, a measure of inflammation
- Albumin, zinc, and calcium levels
- White blood cell levels
- Liver enzyme levels
- Antistreptolysin antibodies, which help fight group A streptococcus, the bacteria that cause strep throat
- Lipid, or blood fat, levels
To rule out an infection that's causing your symptoms, your doctor may also want to check the pustule, and your blood and urine, for bacteria.
A skin biopsy also can help to diagnose GPP and is still recommended because it could rule out other disorders. To do a biopsy, your doctor will take a small skin tissue sample to be tested in a lab.
Genetic testing for GPP
While it's not routine, if you developed GPP at a young age, have recurrent or several flares, or have pustular disease without typical plaque psoriasis, your doctor may recommend genetic testing.
Finding Expert Care
A dermatologist should oversee care for your generalized pustular psoriasis. Dermatologists specialize in skin conditions. Your primary care doctor should also be aware of your GPP and involved in your care. Emergency room or urgent care doctors often play a role as well, if you need urgent care because of a flare.
If you're worried you have GPP or have been diagnosed, it's important to find a dermatologist experienced in treating the condition. If you can't find a GPP specialist close to home or travel for an appointment, ask your dermatologist if they'd be willing to consult with another doctor who has more experience diagnosing and treating GPP. The National Psoriasis Foundation has a Health Care Provider Directory, where you can find specialists in various locations.
Other specialists who may be part of your care team include:
- Immunologists and allergists, who can help manage the involvement of your immune system in GPP or identify triggers
- Rheumatologists, who treat arthritis, a common complication in people with GPP
- Psychiatrists, who can help manage your emotional and mental health as you live with this chronic condition
- Geneticists and genetic counselors, if your GPP may be linked to an inherited mutation in a gene like IL36RN
Questions to Ask Your Doctor
You and your doctor will discuss your diagnosis and care in detail. Some good questions to ask include:
- What's the right medication for me and how soon will it start to work?
- What options are there if my medication does not work?
- How can I prevent flares?
- How can I identify what triggers my flares?
- Can lifestyle changes, such as diet, help?
- What should I do if I have a flare?
- When should I call my doctor or go right to the hospital?
- What should I do if a flare starts while I am traveling?
What Are My Treatment Options for Generalized Pustular Psoriasis?
Your treatment will depend on how severe your disease is. If your GPP was caused by a medication that you take, your doctor will have you stop taking it, if possible.
If you have severe GPP, you'll receive treatments to get flares under control. After that, you'll likely need ongoing treatment to help prevent future flares. You also may need to be hospitalized when you first get symptoms or when you have a flare, particularly if your blood pressure, heart rate, body temperature, or other vital signs are concerning. While you're in the hospital, your doctor will monitor you closely for any potentially life-threatening complications, such as sepsis.
Medications that treat generalized pustular psoriasis
Several medications that target your immune system can help ease flares and prevent them from recurring. The newest, approved by the FDA in 2022, is a biologic called spesolimab-sbzo (Spevigo). It's the first drug to target an immune system protein called the IL-36 receptor. This medicine has been shown to quickly stop flares and prevent recurrences of GPP. The recommended dose for a flare is 900 mg, given by IV, and this can be repeated one week later if you still have symptoms.
If you aren't having a flare but your doctor is worried you could, you might be able to take spesolimab prophylactically. In other words, you can use it to reduce your chances of a flare happening. This dose is 600 mg, given by a shot just under your skin (called subcutaneous, or s/c), followed by a 300 mg dose four weeks later, and then every four weeks after. You can take this at home after your doctor shows you how.
Other medicines commonly prescribed for GPP include:
- Infliximab (Remicade), a TNF alpha inhibitor delivered intravenously
- Cyclosporine, an immunosuppressant typically given for short periods because of its serious side effects from long-term use
- Other biologics, including secukinumab (Cosentyx) and ustekinumab (Stelara)
If your GPP is milder or you're taking medicine mainly to prevent a future flare, your doctor may recommend acitretin (Soriatane) or methotrexate (Rheumatrex and other brand names). Both medicines can cause birth defects, so talk to your doctor before taking them if you are pregnant or you hope to become pregnant.
It's not yet known whether it's safe to take spesolimab during pregnancy. If you develop GPP while pregnant, ask your doctor about treatment options, which may include:
- Systemic corticosteroids, although some discourage the use of corticosteroids because of the possibility of flare
- Cyclosporine
- Antibiotics
- Narrow-band ultraviolet B light therapy
Your doctor may prescribe topical medications if the systemic medicines you take don't fully clear up your skin. These include:
- Topical corticosteroids
- Topical vitamin D analogs
- Topical tacrolimus (Protopic)
Along with prescription topical medications, moisturizing creams, oatmeal baths, and wet wraps also can help to relieve skin symptoms.
Advances in treating GPP
Researchers are looking at other drugs and if they are effective in treating or preventing GPP flares. For example, imsidolimab is currently under study and showing promise in clearing up skin among people who have moderate to severe GPP flares.
Several medicines, such as adalimumab (Humira), are approved for use for GPP in Japan, but not in the U.S. But they are approved to treat other conditions in the U.S., and sometimes doctors may prescribe them off-label for GPP. These other medicines may include:
- Brodalumab (Siliq)
- Certolizumab pegol (Cimzia)
- Guselkumab (Tremfya)
- Infliximab (Remicade)
- Ixekizumab (Taltz)
- Risankizumab (Skyrizi)
- Secukinumab (Cosentyx)
What to Expect When You're Living with Generalized Pustular Psoriasis
GPP is a chronic, unpredictable, and potentially life-threatening disease. That sounds scary, but there are things you can do to take care of yourself, both during a flare and between flares.
Tips to help yourself during or between flares:
- Stay hydrated by drinking plenty of fluids.
- Keep your skin moisturized.
- Try to stay calm.
- Explore meditation, yoga, and other relaxing activities.
- Make rest and sleep priorities.
- Ask for help from friends and family.
- Stick to your treatment plan.
- Wear sunscreen and limit the time you spend in the sun.
- Stay physically active.
- Curb the amount of alcohol you drink.
- Eat a healthy diet.
- Quit smoking.
How Does GPP Change Over Time?
Generalized pustular psoriasis is a chronic condition. That means it never entirely goes away. You can expect the disease to flare up from time to time. Usually, the flares will go away after a few weeks, although it can take longer.
Your doctor won't be able to predict how GPP will affect you, as it varies from person to person and over time. You could have several flares a year, or you may have a flare only every few years.
Your flares may also be different each time. Some may be severe, causing widespread skin problems as well as harmful symptoms like inflammation throughout your body. Other flares may be a lot milder, causing no symptoms of serious concern. An estimated 50% of flares lead to a hospital stay. It's likely you'll be hospitalized for a flare at least once every five years on average.
For most people with GPP, skin symptoms don't entirely clear up between flares. But 9 out of 10 people with GPP respond to medications that get their flares under control.
What Are the Complications of Generalized Pustular Psoriasis?
GPP flares can be life-threatening. Potentially fatal complications include:
- Sepsis
- Heart failure
- Kidney failure
- Liver failure
- Respiratory failure
Other serious complications that can occur during a GPP flare include:
- High fever
- Low blood pressure
- Swelling in the legs
- Uveitis, a type of inflammation in the eyes
- Arthritis
Where Can I Get Support for GPP?
People living with GPP often need practical, emotional, and logistical support, especially during flares that interfere with daily activities. You may need extra support or assistance with walking, dressing, cooking, or running errands.
You can start by creating a "support circle" of trusted family, friends, or neighbors who can step in when needed. They might do specific tasks such as grocery shopping, giving you rides to appointments, or helping you with your children on bad days.
While everyone is different, you may benefit from connecting with patient organizations and local support communities, although it can be more challenging for a rare disease like GPP. Reaching out to the National Psoriasis Foundation is a good first step.
If you can't find enough support locally, look for support groups or other resources online or via social media channels. Check to see what your local clinic or insurance company offers in terms of resources and coverage for social workers, mental health care, or other forms of personal support, if needed.
Caring for Your Mental and Emotional Health
Having GPP can be stressful, to say the least. Stress is also a major trigger of GPP flares. Finding ways to manage your stress – and other aspects of your mental and emotional health – is critical. Your doctor can help with referrals to counseling, medication, and other resources. Also, try the following:
- Practice mindfulness and meditation with the help of an app.
- Establish a yoga or light exercise routine.
- Find relaxing activities that you enjoy, like reading, listening to music, or watching a funny movie.
- Spend time with friends and family, which can help to improve your mood.
It can also help to reach out to others with GPP. The National Psoriasis Foundation offers both online and in-person ways to be part of the support community. Ask them about any groups or resources specific to GPP.
Information for Caregivers
Caring for someone with GPP can be demanding and overwhelming, not only when they're living with a flare, but in between flares. You might spend a lot of time worrying about when the next flare might happen.
When your loved one is in remission, you can help by encouraging rest, hydration, and generally practicing good self-care. You can also watch for warning signs of a flare, like the sudden appearance of pustules, fever, or chills. If you notice any signs of a flare, seek medical help right away.
When a loved one has a chronic illness, it takes a toll on caregivers, too. It's important for you to take care of yourself first. Take breaks and ask for help. You can also reach out for help from the National Psoriasis Foundation.
Takeaways
Generalized pustular psoriasis is very rare but very serious, even life-threatening. If you develop this disease, follow your treatment plan carefully to get your flares under control and to help prevent future flares. Call your doctor at the first sign of symptoms even if you normally have mild flares. The disease is unpredictable, and any flare can become quite serious.
Generalized Pustular Psoriasis FAQs
How serious is generalized pustular psoriasis?
Very serious. A GPP flare can be life-threatening.
What is most likely to trigger a generalized pustular psoriasis flare?
The most common triggers of GPP flares include stress, infections, stopping a corticosteroid medication, and pregnancy.
How can you stop pustular psoriasis from spreading?
The biologic medicine spesolimab has been shown to stop flares quickly. Other medications also can be effective when you have a GPP flare.

