Your FAQs around generalized pustular psoriasis

Medical News Today

3 days ago

Generalized pustular psoriasis is a severe form of psoriasis that can cause body-wide symptoms and may require immediate medical attention.Generalized pustular psoriasis (GPP), also known as von Zumbusch psoriasis, is a rare type of psoriasis.Globally, GPP affects an estimated 1 to 7 people out of every million. It features episodes or 'flares' of erythroderma, widespread skin inflammation and redness or other skin color changes. These flares develop with sterile (not caused by infection), fluid-filled blisters or pustules.During a flare, pustules break open, and the skin can become dry and painful. It may crack, peel, or scale and may resemble a burn.GPP is an immune-mediated condition caused by dysfunction in the immune system. Unusual immune responses to triggers such as stress, medication use, or infections can set off a chain of severe inflammatory reactions throughout the body. GPP often has identifiable triggers, but it can also happen without any obvious cause.Many people experience severe systemic symptoms in addition to skin inflammation, including fever, chills, muscle weakness, and fatigue. If left untreated, GPP can cause life threatening complications like organ failure.Read on to find out the answers to some of your most pressing questions about GPP.What is the outlook for generalized pustular psoriasis?GPP is a chronic condition. It can be managed, but it may never be fully cured. As many as 80% of people experience relapses, and most have at least one episode yearly.The outlook during a flare depends largely on how quickly a person receives treatment. Severe inflammation can cause tissue damage and affect internal organs like the heart, lungs, or kidneys. In some cases, this may result in sepsis, a life threatening complication that requires urgent care. Seeking treatment as soon as possible can help shorten the duration of a GPP flare and lower the risk of serious complications.Even between flares, GPP can affect long-term health. People with GPP have a higher risk of cardiovascular conditions and other systemic complications, which can affect quality of life and life expectancy.According to a 2025 study, people with GPP have about four times the risk of death from any cause compared to the general population.What is the best treatment for generalized pustular psoriasis?Everyone living with GPP has different factors that can affect their treatment plan. A doctor will consider a person's age, family history, genetics, and other health conditions when recommending treatment.During a flare, first-line treatments usually include medications that target the immune system. These drugs work by blocking or calming overactive inflammatory pathways.One example is spesolimab (Spevigo), a monoclonal antibody that blocks interleukin-36 (IL-36) receptors. IL-36 is a pro-inflammatory molecule, known as a cytokine, that becomes overactive in GPP. It triggers systemic inflammation and stimulates the production of neutrophils, which accumulate in the skin and create pustules.Currently, spesolimab is the only medication approved by the Food and Drug Administration (FDA) specifically for the treatment of GPP.Doctors may also use other medications that modify immune responses or control inflammation, such as:infliximaboral retinoidsapremilastmethotrexatecyclosporineFor mild symptoms or when GPP is well managed, doctors may recommend more conservative treatments. These can include keeping the skin hydrated, using topical corticosteroids, and prescribing antibiotics if there is an infection.If a medication triggers the flare, stopping that medication is also an important part of the treatment.How do you stop pustules from spreading?Immediate treatment helps stop GPP pustules from spreading.Pustules in GPP are sterile. They form because of overactive inflammatory processes in the body. Itching pustules or having pustules break open is not what causes pustules to spread (although this can increase the risk of secondary infection).Interrupting the inflammatory chain with medications like spesolimab during a flare will help bring pustules under control. Applying cool compresses, keeping skin clean and moisturized, and avoiding irritants like rough fabric can help improve the overall health of the skin and manage discomfort.Can pustular psoriasis lead to sepsis?Generalized pustular psoriasis can lead to sepsis.Sepsis is a severe, life threatening inflammatory reaction to an infection that spreads through the bloodstream. It can cause symptoms such as: feverrapid heartbeatrapid breathinga dangerous drop in blood pressureconfusionorgan damageIn GPP, several factors can increase the risk of sepsis. Open skin from pustules, cracks, or scratching allows bacteria, viruses, or fungi to cross the skin barrier and enter the bloodstream. Medications that suppress the immune system, such as methotrexate or infliximab, can also make it harder for the body to fight infections.Once these pathogens enter the bloodstream, they can travel throughout the body, triggering a widespread inflammatory response known as sepsis.Does sepsis happen during every generalized pustular psoriasis flare?Sepsis can occur during a flare, but it does not happen every time a person with GPP experiences a flare.Flares in GPP are believed to be caused by a 'cytokine storm.' A cytokine storm happens when the immune system becomes overactive and releases too many pro-inflammatory molecules at once. Infection can trigger a cytokine storm, but it is not the only cause. Cytokine storms in GPP can happen any time the immune system is overactivated, even without an infection.Sepsis, however, always results from an infection. It also involves a cytokine storm, but in this case, the overactivation of cytokines is a direct response to that infection. In conditions like GPP, existing cytokine dysfunction can make a sepsis-related cytokine storm more severe.SummaryGeneralized pustular psoriasis is a rare form of psoriasis that causes widespread areas of red, inflamed skin covered with sterile, fluid-filled pustules. GPP can be life threatening. It causes severe, body-wide inflammation that may damage tissues and lead to organ failure.Prompt treatment can help slow the spread of pustules and lower the risk of serious complications. Doctors typically treat GPP with medications that interrupt or block overactive immune responses. Spesolimab (Spevigo) is the only drug currently approved by the FDA specifically for the treatment of GPP.