Coming off cortisone too quickly can trigger the rare skin condition in psoriasis patients.
People with generalised pustular psoriasis flares are more than twice as likely to visit the emergency department than patients without flares, a study has found.
Generalised pustular psoriasis is a rare sub-type of psoriasis and can be so severe that patients need to be treated in hospital.
The condition is characterised by the sudden formation of pustules along with systemic symptoms such as fever and chills.
The US study looked at flares among people with generalised pustular psoriasis and the differences between patients with and without flares, using US health records.
Patients with documented flares were almost three times more likely to have any inpatient visits (119 of 271, or 44%) compared to patients without flares (194 of 1264, or 14%). They were twice as likely to have any emergency department visits than patients without flares, the study found.
“Generalised pustular psoriasis flares can be severe, often requiring emergency department care or acute care,” the authors wrote in JAMA Dermatology.
“Despite this high level of severity, advanced treatments are very rarely used during flare episodes, leaving a significant unmet treatment need for patients with GPP and for health care professionals.”
The retrospective observational cohort study over five years identified patients through US electronic health records of 48 million people.
Of those, 1535 patients with generalised pustular psoriasis were identified, two-thirds of them women. Among that group, 271 patients had at least one flare, with a total of 513 flare episodes. The mean follow-up time was 724 days.
Patients with documented flares had a higher comorbidity than those without flares, they found.
A third of patients were prescribed topical corticosteroids, which was the most common treatment. Pain was the most common symptom, and one in five patients were prescribed opioids.
“However, despite the severity of the flare episodes, topical corticosteroids were the most common treatment during flares, and use of biologics or similar advanced treatments was low,” the authors wrote.
A quarter of patients were not given any additional treatment in the month before the flare, during, or in the month after the flare, the authors wrote.
Generalised pustular psoriasis was rare in Australia, said Sydney-based dermatologist Associate Professor Stephen Shumack.
“Most of the cases we see, and even they are rare, are caused by stopping oral steroids such as prednisone too quickly,” he said.
“If rheumatology or gastroenterology patients also have psoriasis, and come off oral steroids too quickly, then you can make their ordinary psoriasis turn into pustular psoriasis. But this is very uncommon.
“This is one of the reasons why we don’t generally use oral steroids in people with psoriasis, because if you stop it quickly, you can get quite a nasty pustular flare.”
It could also be triggered by pregnancy or other medications, said Professor Shumack, senior staff specialist at the Royal North Shore Hospital of Sydney and clinical associate professor at the University of Sydney.
Advanced therapies such as biologics took a few months to take effect and did not work fast enough to treat immediate flares, he said.
“They don’t work overnight, and you want a flare in pustular psoriasis settled fairly quickly,” Professor Shumack told Dermatology Republic.
“Even the IL-17s, which are pretty fast and work in two to four weeks, are not fast enough in these flares, because these patients are feverish and are feeling unwell so you want them fixed pretty quickly.”
Professor Shumack said there were significant differences in the patients reported in the retrospective, chart analysis study compared to patients normally seen in Australia.
“In Australia, we would not expect the advanced therapies to be used for an acute flare because the medications and management strategies that we have usually bring the flare of pustular psoriasis under control very quickly,” he said.
“We usually admit them to hospital for topical steroids and wet dressings soaked in water to aid in the penetration, maybe going back on a small or medium dose of prednisone to taper it down if that precipitated it, and ciclosporine for a short period of time if it was really severe. And they work in a matter of days or a week.
“However, if the patient remains with moderately severe, or severe pustular psoriasis, we would consider the use of advanced therapies such as biological agents through the PBS as we would in other patients with severe psoriasis.
“We tend not to use methotrexate or mycophenolate unless there’s a particular reason we would use those. Generally we’d use ciclosporin because it works much faster.”
Professor Shumack said he was surprised that opioids has been prescribed so frequently in the US study, and in 30 years of practice he had not prescribed opioids to patients with the condition.
The US study included lower socioeconomic groups which may be a more severe cohort with later presentation, he said.
Professor Dedee Murrell, head of the department of dermatology at St George Hospital and professor of dermatology at UNSW, told Dermatology Republic that generalised pustular psoriasis was more common among people with darker skin such as people of African descent.
As the condition was so rare in Australia it could easily be missed by GPs, who may diagnose it as eczema and prescribe steroids, Professor Murrell said. Not all patients were referred to specialists, she said.
“Patients get treated with steroids tablets, but the steroids give them diabetes, high blood pressure and fractures. There’s a whole host of side effects, but patients don’t often realise what the long-term consequences of being on steroids are,” she said.
“That’s why more specific treatments such as biologic treatments, which block the specific pathway that’s increased in the disease, are safer treatments.”
A study published in the NEJM found that some patients with generalised pustular psoriasis had a genetic mutation of the IL36RN gene, paving the way for therapeutic interventions.
The researchers found that a single dose of a monoclonal antibody against the interleukin-36 receptor reduced the severity of generalised pustular psoriasis over 20 weeks.
“This new treatment, which has only been studied in a limited number of people so far, is looking very promising,” Professor Murrell said.
Patients with severe skin diseases may cover up their skin out of embarrassment, Professor Murrell said.
“These patients had often given up and they would cover up their skin disease with their clothes. And they would go around feeling terrible, but not telling anyone that they had a problem because they’d hide it under their clothes. And some of them would commit suicide,” she said.
“There may be patients who are hidden in the woodwork hiding away thinking there’s no treatment, and they’re not aware that there could be a treatment.
“Sometimes they get in that minds that they’ve tried so many things, no one cares that they have these problems or that treatments exist, and their GPs are none the wiser.
“When it’s very rare, GP don’t know what to do, patients give up, and they become recluses and hide away from society. Just because we’re not aware of them, doesn’t mean they’re not there.”
