KUALA LUMPUR, Jan 31 — For 35 years, Joyce Choo has lived with the burden of generalized pustular psoriasis (GPP). Diagnosed at age 11, she recalls how her condition progressed from erythrodermic psoriasis to GPP, one of its rarest and most severe forms.
“At first, it was erythrodermic psoriasis,” Choo shared at a launch and roundtable discussion on a GPP 2024 report by the Galen Centre for Health and Social Policy last November 19, supported by Boehringer Ingelheim (Malaysia) Sdn Bhd.
“I always ended up hospitalised because the GPP flares were terrible — burning sensations, swelling, high fever, and pustular lesions covering me from head to toe, including my scalp, palms, and soles of my feet. I couldn’t walk and had to rely on a wheelchair.
“Every time it happened, I would stay in the hospital for a minimum of two weeks. I was very, very sick because the fever would reach up to 40 or 42 degrees Celsius. Even with paracetamol, the temperature wouldn’t come down.”
The aftereffects of a flare brought its own challenges. “Once the pustules dried, my skin would become very sensitive and tender because the first layer had peeled off. It had an unpleasant smell. I also had difficulty sleeping because it was very, very painful,” she said.
Her physical symptoms were only part of the ordeal. “My self-esteem is always low. The hospital is my second home. I don’t have many close friends because of my appearance. People would stare at me like I was an alien, so life has been very challenging.
“Also, because my GPP was very unstable, it affected my further education. I couldn’t enrol in university, and I was very sick. GPP has influenced and limited my life in so many ways,” Choo added.
GPP Prevalence In Malaysia
GPP is a rare and severe subtype of psoriasis characterised by its debilitating symptoms. Globally, it affects roughly one in nine million people, but its prevalence in Malaysia is notably higher.
According to a policy report by the Galen Centre, a 10-year cohort study in Johor found a prevalence of 198 cases per million, with a higher incidence among women and individuals of Chinese ethnicity.
Dr Choon Siew Eng, an associate professor at Clinical School Johor Bahru, Monash University Malaysia and visiting senior consultant dermatologist at Hospital Sultanah Aminah, Johor Bahru, noted that the higher prevalence among women is likely explained by the trigger factors.
“We know that GPP flares can be triggered by pregnancy and menstruation, which may explain the higher prevalence in women,” Dr Choon said.
“Based on our study on GPP patients and international studies, the IL36RN mutation is certainly more prevalent in Asians than in Caucasians. Even among our local population, we see a higher prevalence of this mutation in Chinese individuals.”
The IL36RN mutation affects a protein that controls skin inflammation. In GPP, this mutation triggers excessive immune responses, causing pustules, redness, and pain.
GPP Rare, But Severe Skin Condition
Dr Azura Mohd Affandi, a consultant dermatologist at Kuala Lumpur Hospital and the national head of service for dermatology, described GPP as one of the more challenging dermatological conditions to manage.
“GPP stands out because of its unpredictability,” Dr Azura said. “Patients don’t have control over when they’re going to have a flare. When they do have a flare, it’s usually quite severe, and they need to be admitted.
“They don’t always have to be admitted if the flare is mild, but most of the time they do because we need to perform intensive skin dressing to manage the flare and reduce the pain.
“In some patients, the pain is so severe that we have to refer them to the Acute Pain Service at our hospital. If you can imagine, they are covered in pustules, and once the pustules rupture, they develop erosions. It’s a very painful condition, and these erosions stick to bedsheets and clothing.
“So simple things we take for granted — like bathing or even just washing their face — become difficult because of the pain they endure.
“In terms of severity, it’s one of the most serious skin diseases we see in our clinic. Of course, there are other severe conditions, such as allergies like Stevens-Johnson Syndrome and toxic epidermal necrolysis. We also see autoimmune skin diseases where patients require admission.
“So GPP, I would put it on par with these diseases due to its severity, unpredictability, and high rates of hospital admission,” Dr Azura said.
Choo described the immense pain caused by GPP, rating it a 10 out of 10. “It’s not just the skin pain; I also have joint pain, which comes with swelling and movement limitations. My ankle swells, and I sometimes struggle to move.”
Public Awareness Of GPP Still Lacking
Choo shared that, beyond physical pain, patients also suffer from mental health challenges and stigma. “Psychologically, patients often lack strong support. I don’t have many close friends because people also worry about whether the disease is contagious. Thirty years ago, awareness was very limited. There was no internet, and social media wasn’t as strong, so no one knew about psoriasis. Even my class teacher didn’t know what it was,” she said.
Sofia Lovi Ramasamy, interim president of the Psoriasis Association of Malaysia, said that awareness of GPP is still lacking compared to other more common forms of psoriasis, which, though unfamiliar about 30 years ago, is now more widely understood.
“The word ‘psoriasis’ has existed for many years, but familiarity with it is relatively recent. Thirty years ago, people didn’t know what psoriasis was. Today, people are more aware of it, but GPP is still not widely recognised.
“Yes, we’ve made progress in familiarising people with the term ‘psoriasis’ — we can check that box. But GPP is still not well-known because it’s considered rare. Based on the data, the statistics don’t show many patients being diagnosed, and we don’t have accurate figures on how many people in Malaysia are living with it,” said Sofia, who is also living with psoriasis.
“I think we need more GPP patients coming up and telling us more stories, sharing life experience is so important, so that the people out there will be able to know what the real sufferings are. If we want to eliminate social stigma, what steps need to be taken?
“And if I can add another point, while dermatologists focus on the outer part of the skin and work to ease our physical pain, what about the emotional aspect?
“The treatment isn’t yet at a stage where it heals us internally. Yes, the skin may look good, we may appear fine on the outside, but emotionally, we’re still struggling. That part hasn’t been addressed adequately,” Sofia said.
Rare Disease Patients and Caregivers Often Crowdfund For Treatment
Nadiah Hanim Abdul Latif, president of the Malaysian Rare Disorders Society, shared that many families dealing with rare diseases often have to turn to crowdfunding to finance treatment.
“It’s not because that’s what they want, but because of the urgency of time and criticality of needs. Sometimes you have to put your face aside and go out there to ask people for funds. It’s not an easy thing to do,” Nadiah said.
“For a lot of families looking at enzyme replacement therapy or exome sequencing, you’re talking about upwards of millions of ringgits. While this might work for a while, we do worry that it might not be a sustainable method to crowdfund for everyone, as it could become an equity issue and a donor-fatigue issue.
“We also know that in the current climate, there’s a dwindling amount of CSR (corporate social responsibility) funds. Most of you would have written to corporate companies, asking for yayasan (foundation) funding or CSR funds, and you will find that it’s harder now to get funds from corporate entities than before, as times are getting tougher.”
One area Nadiah is focused on is the Income Tax Act 1967, which, she explained, currently allows funds to be used for certain charitable purposes but not necessarily for rare disease treatments.
“Because of this, funds often go toward helping orphanages or road repairs, not necessarily for the causes we need. There are legislative changes and amendments needed to create more options for us moving forward,” she said.
“That said, we do see a lot of companies contributing, but their focus tends to be on awareness rather than direct patient treatment,” Nadiah added.
Overcoming Financial and Policy Barriers To GPP Treatment
The Galen Centre’s policy report noted that despite nearly one in four GPP patients being classified as having moderate to severe disease, the use of biologic therapy remains limited, indicating delays in treatment escalation.
Dr Mohd Azman Yacob, director of the medical development division at the Ministry of Health (MOH), highlighted the importance of improving treatment outcomes and early diagnosis for rare disease patients. This includes the need for a clear policy from the MOH on rare diseases to overcome funding constraints.
“The most important thing for the MOH is to have a clear policy on how we’re going to address this issue at the macro level, for rare diseases and specifically for GPP.”
Dr Mohd Azman also spoke about the importance of a national programme to improve access to innovative treatments for rare diseases, as well as increased budget for drugs that are already on the formulary.
“By having a national programme, we can hopefully improve our access to biologic therapies – this is the most important. Some biologic drugs are already in our FUKKM (MOH’s Medicines Formulary), but from time to time, we need to update our selection of drugs. Once the drugs are in FUKKM, we have to ensure we provide the necessary budget for them,” he said.
