Managing Uncertainty with GPP

Both disorders require different treatment. About 50% of people with GPP also have plaque psoriasis, according to the National Organization for Rare Disorders.

A 2022 study found that people with GPP reported more pain, fatigue, and itching than those with plaque psoriasis. They also reported more treatment changes, a sign that current treatments were not working well enough.

GPP also occurs in a cycle. That is, the pustules can break open. When they do, they leave pus on the skin – something called “lakes of pus.” When the skin dries out, you may see a reddish tone on lighter skin or a purplish tone on darker skin. The skin then flakes and sheds off. 

Causes of GPP

The following can set off symptoms of GPP: 

• Stopping a medication
• Infections (such as COVID-19, chickenpox, strep throat, and shingles)
• Stress
• Injuries
• Vaccines
• Low calcium (hypocalcemia)
• Sunlight
• Smoking
• Menstruation
• Pregnancy
• Genetic changes in the IL36RN and CARD14 genes

GPP Diagnosis

Quickly diagnosing GPP may prevent you from having severe complications or death from other conditions such as acute kidney failure, sepsis, and others. 

A 2022 survey on Americans living with GPP revealed that 36% of those in the study lived with symptoms for months before diagnosis and 38% lived with symptoms for years before a formal diagnosis. Of the people in the study, 59% were misdiagnosed and 51% had to see multiple health care professionals. 

Your doctor will look at the rash and may take a biopsy or sample of your skin. A biopsy involves shaving off a small amount of the skin, punching it to get a sample of cells beneath the surface, or cutting a small piece out of your skin. Your doctor can numb the area so the procedure is less painful. Your doctor may also order a blood test.

Your doctor will ask about other symptoms you may have had such as swelling, pain, fever, fatigue, body aches, and headaches.

GPP on different skin tones

This skin disorder can appear differently based on the color or tone of your skin. On lighter tones, it may look reddish. The skin color may not change in darker tones, but there may be a purplish tone or darker tone. 

GPP Treatments

The treatment your health care provider recommends will depend on whether you have mild or severe GPP as well as your long-term goals. Many people need ongoing treatment to lower the chance of flare ups and/or stay in remission.

Spesolimab (Spevigo) is the only biologic drug approved by the FDA to treat GPP. You may receive it via an IV or an injection. Your doctor may prescribe these other medications for GPP:

• Apremilast (Otezla)
• Cyclosporine (Neoral)
• IL-23 inhibitors like guselkumab (Tremfya)
• Infliximab (Remicade)
• Ixekizumab (Taltz)
• Oral retinoids like acitretin (Soriatane) 
• Methotrexate (Otrexup)
• Secukinumab (Cosentyx)
• Topical medications including corticosteroids and nonsteroidal drugs

Non-medication treatments may include psoralen plus ultraviolet (PUVA) photochemotherapy. This involves taking medication and exposing your skin to ultraviolet A light. 

Who Gets GPP?

Globally, about 2 to 7 million people have GPP. Pustular psoriasis makes up approximately 1% of all clinical cases.

GPP can start at any age but often occurs between 40 and 60 years old. Women and people assigned female at birth are more likely to get it than men and people assigned male at birth. Some people can get GPP during the third trimester of pregnancy. It usually clears up after childbirth.

Dangers of a GPP flare

A GPP flare can be serious. It causes systemic inflammation that can lead to serious problems if not treated.

A 2024 analysis of 30 studies on GPP showed that people with the disorder had flares once a year and the flares lasted from two weeks to three months. More than 80% of people had issues with daily activities even after a flare ended. 

Complications can include the following:

• Sudden drop in blood pressure
• Gastrointestinal discomfort
• Delirium
• Tetany (involuntary muscle contractions)
• Cardiac failure
• Renal injury or failure
• Respiratory failure
• Hair loss
• Jaundice
• Hypoalbuminemia (low albumin)
• Sepsis
• Death

A 2024 analysis found that people with GPP were prescribed more medication, had more comorbidities, and used inpatient and outpatient visits than in those with plaque psoriasis and those without psoriasis. Flares often required inpatient hospital treatment, the report showed.

Even when not in flare-up mode, many people with GPP experienced moderate or severe pain when their symptoms were under control, a 2022 report found.

GPP prognosis

There is no cure for GPP. After a flare, it can take weeks or a few months for your skin to recover.

GPP can occur repeatedly during the course of your life. In addition to symptoms that affect the skin, it may make you feel ill with symptoms such as fever, rapid pulse, fatigue, nausea, muscle weakness, joint pain, and headache.

While you may not be able to prevent a flare, knowing what sets off an episode may help you avoid triggers. Keep track of possible triggers in a journal and discuss them with your doctor. Not everyone has identified their triggers, as a 2024 analysis found that anywhere from about 15% to 62% of people with GP are unaware of what sets off a GPP flare.

A type of pustular psoriasis called acrodermatitis continua of Hallopeau, which causes pus-filled bumps on the toes, fingertips, or underneath nails, seems to have the worst prognosis among all types of pustular psoriasis.

As for GPP itself, the prognosis is fairly good so long as you treat the disorder.

How GPP Can Affect Mental Health

When you’re dealing with a disorder that is painful, difficult to disguise, and makes you feel physically sick, it can affect your mental health. On top of that, the fact that it can pop up at any time can add more stress to your life.

One small study found people were concerned about whether or not other people could see their affected skin, felt helpless about their disorder, felt like others avoided them, and didn’t feel they could participate in activities due to their disorder. All of these concerns can impact mental health. 

Other mental health impacts of psoriasis 

The uncertainty of a flare can cause mental health issues, but not being able to work may contribute as well. That same 2022 study found that 20% with GPP were disabled compared to 7.6% with plaque psoriasis.

A 2022 survey on Americans living with GPP showed that emotional stress was the most common cause of flares. Of respondents, 67% had fear and anxiety about their disorder and 71% had a fear of flares. The survey also revealed that 65% feared that their current medication would stop working, 59% said they had feelings of hopelessness and depression, and 56% worried that their GPP would get worse with age. 

Changes in mood was the symptom reported in a flare, and 57% said it carried a high burden of 8 to 10 on a scale of 0 to 10; 43% said that burden was between 5 to 7 on the same scale, the survey found. 

During flares, 58% of respondents said GPP had a high impact on their ability to exercise, 52% said it affected their ability to be intimate with a partner, 44% said it interrupted their ability to complete errands, and 41% said it impaired them from social interactions. Even when not in flare mode, 21% had disruptions exercising, 23% struggled with intimacy, and 15% said it kept them from attending important life events, the survey showed.

In addition to mental health issues, you may be more likely to drink alcohol or smoke if you have psoriasis. Those can make psoriasis worse.

The anxiety and depression link

GPP negatively affected the mental health of people with the disorder, as anxiety and depression are common, a 2024 analysis found. In fact, more people with GPP reported anxiety or depression compared to those with plaque psoriasis and those without any psoriasis.

Strategies to Reduce GPP-Related Anxiety

According to one survey of people with psoriasis in the U.K., 98% of respondents said psoriasis affected their emotional or psychological well-being, but only 18% got help for their mental health issues.

There are many things you can do to ease anxiety and depression, which are common with GPP. It’s important to remember that you’re not alone in these challenges, and there are resources to support you.

Anxiety, namely, can affect you in many ways. For some people, anxiety from GPP impacts their thoughts; others may be in a bad or low mood and have physical reactions. For others, it impacts their behavior. All of these factors can impact the other  and repeat as part of a cycle.

These are a few ways to reduce the anxiety you may feel with GPP:

• Talk to a trusted friend or family member.
• Join a support group or seek out therapy.
• Prioritize your sleep schedule.
• Have an action plan in place if you have a flare.
• Practice stress relief techniques such as mindfulness, breathing, and exercise.
• Learn ways to combat negative thinking.
• Actively combat stigma by educating others on the disorder.
• Join a clinical trial.

How to Prepare for Emergencies

When you know what symptoms could be serious, you can be better prepared to tell if you need emergency care. If you have any of these symptoms in addition to pustules on your skin, visit your local emergency room:

• Fever
• Muscle weakness
• Severe pain
• Itchiness

Takeaways

GPP is a complex disorder that affects your body and can impact your mental health. You are more prone to anxiety and other mental health issues because of GPP, so it’s a good idea to find support in a way that works for you.