KUALA LUMPUR, Nov 10 – Patients with generalised pustular psoriasis (GPP) in Malaysia often suffer in silence, as their condition is rare and extremely severe, but it’s often misdiagnosed as an infection.[4]
GPP – a subset of psoriatic diseases, reported at one per 100,000 cases of psoriasis[4] – isn’t contagious. More than a skin condition, GPP, an autoinflammatory disease, can lead to heart failure, kidney failure, and life-threatening infections in the long run.[4]
The rare and severe form of psoriasis is characterised by recurrent episodes of widespread, sterile, and macroscopically visible pustules. Patients usually experience “flares” – potentially lethal episodes of painful and red skin – which can happen several times a year or over a longer period.[4]
“The uncertainty of diagnosis or misdiagnosis leads to prolonged time of receiving accurate diagnosis which further extends the point of treatment initiation.
“Patients are then left with no option but to either endure the discomfort and pain experienced, or to opt for alternative treatment which does not have strong clinical evidence to treat GPP flares,” wrote the Galen Centre for Health and Social Policy on the discussion session of an expert meeting on GPP that it hosted last January 27, with support from Boehringer Ingelheim.[3]
GPP significantly affects one’s quality of life even during the dormant phase for patients in Malaysia[4]. Psoriasis patients, including those with GPP, also report feeling guilt, resentment, fear, and difficulties with daily tasks and social interactions, worsened by the severity of GPP.
Besides misdiagnosis, there are limited options for treatment of GPP, limited insurance coverage, and stigma and discrimination against people with the condition in Malaysia.[4]
GPP Most Frequently Diagnosed in Middle Age
GPP is most often diagnosed in middle-aged adults.[4] The prevalence of GPP in Malaysia – at one per 100,000 cases of psoriasis – is reportedly believed to be higher than other countries.[4]
In Malaysia, the most frequently reported subtype was acute GPP, detected in many psoriasis patients in one study (Choon et al., 2014),[1] and comprising 56 per cent in another study (Zelickson & Muller, 1991).[11]
Data from Malaysia indicate that individuals with adult-onset GPP had disease onset at an average 41 years old, ranging from 21 to 81 years old. Other studies indicate the mean age at diagnosis ranging from 45.6 to 50 years old (Zelickson & Muller, 1991).[11]
While GPP usually affects senior citizens in Malaysia, the Galen Centre noted that it’s also possible to find cases of young people with the condition.[4]
An all-over generalised pustular eruption is the feature of a GPP flare that starts out acutely most of the time. Additional symptoms of GPP flares include fever, chills, severe pain, loss of appetite, feeling sick, and feeling tired and generally unwell.[4]
There is no known cause of GPP, but the most common causes of GPP flares include faulty genes, taking some medications or suddenly stopping steroids, hormonal changes and pregnancy, periods of stress and anxiety, infections (for example, dental and throat), and too much sunlight.
Research by Choon et al. (2014)[1] showed that family history was a “powerful predictor” of an earlier onset of psoriasis and pustular psoriasis. People with family history of psoriasis often exhibit their first symptoms nearly a decade, or 9.5 years, earlier than those without family history.
Average One Hospitalisation In Five Years During Flares
Severe GPP treated with systemic treatments frequently involves an inpatient hospital stay during flares. A Malaysian study showed a mean of one hospitalisation for each GPP patient in a follow-up period of about five years (Choon et al. 2014).[1]
During the non-flare period, GPP patients still need to spend money by following up with dermatologists and other doctors and receiving up-to-date and systemic treatments for the management of skin lesions and the prevention of flare recurrences (Choon et al., 2022).[2]
A US study (Sobell at al. 2021)[9] showed that patients with GPP were more likely to have comorbidities, like psoriatic arthritis and hyperlipidaemia, than those with plaque psoriasis. Patients with GPP also used more medications and had higher health care resource utilisation.
Even in the dormant stage, GPP patients in Malaysia experienced a significant decrease in their quality of life (Choon et al., 2014).[1] During follow-up appointments, patients with acute GPP had a mean Dermatology Life Quality Index (DLQI) score of 12.4 points, suggesting severe impairment.
Dermatologist Highlights Treatment Gap for GPP
There exists a gap between disease prevalence and access to efficient biologics in Malaysia when it comes to GPP, said Dr Azura Mohd Affandi, a consultant dermatologist with Kuala Lumpur Hospital (HKL).
“At the moment, we are treating GPP just like plaque psoriasis. We should not be doing this because their clinical presentation is different, but somehow our way of managing patients is like how we treat our plaque psoriasis patients,” Dr Azura told CodeBlue.
“There is a treatment gap because what we are seeing with all this treatment is, when we give steroid-based treatments, the pustules dry up faster. But steroids’ long-term effect is not good. It can make the condition unstable. This means at times; it may not be a good choice. So, there is a treatment need for something that is efficient, that can work fast, and at the same time it’s safe for the patient.”
Dr Azura further lamented that support and awareness for GPP in the country are also not encouraging. There is a support group for psoriasis patients, which is the Psoriasis Association of Malaysia, but it caters more for patients with plaque psoriasis.
“Patients with plaque psoriasis have different symptoms, therefore different presentation. In terms of reading material for GPP, it is definitely not adequate even at the local level. There is a need for proper education materials that the patient can refer to. Many patients refer to the internet, but most sources found are international rather than local.”
But Dr Azura remains optimistic and hopeful about the current local R&D landscape for GPP.
Lack of Biologics in Public Hospitals
GPP requires immediate treatment upon exposure to a flare as it could potentially be life-threatening due to uncontrolled progression[4]. Currently, patients are treated based on their disease profile which subjects each patient to different drugs based on varying drug release durations.
The January expert meeting organised by the Galen Centre noted that the lack of effective treatment in public hospitals limits the timeliness and effectiveness of clinical outcomes for GPP patients[3].
“Biologics have been proven to possess greater efficacy levels; however, these drugs cost much more than the registered counterparts and are not readily available at all medical fraternities. The risks associated with unlicensed drugs such as biologics instil fear and insecurity amongst patients to consume said drugs.”[3]
There is a lack of approved biologics for the treatment of GPP flares worldwide.[3]
Reducing symptom severity and preventing pustule recurrence are the primary goals of GPP treatment in Malaysia. Possible therapies include topical steroids, oral or topical retinoids, phototherapy, and systemic medications such methotrexate, cyclosporine, infliximab, and biologics (Neuhauser, et al., 2020).[7] Moreover, patients may be counselled to alter their lifestyle, such as by avoiding irritants and allergens, using moisturiser, and reducing stress.[4]
“GPP can be effectively managed with the right care, enabling patients to lead regular lives,” the Galen Centre wrote in a White Paper on GPP[4].
The think tank noted that dermatologists consider a variety of different aspects in treating GPP, such as the intensity of the flare and the patient’s risk factors, to decide which course of treatment may be suitable for a certain patient.[4]
“A variety of medications, including some creams, ointments, pills, and injections may be prescribed to a patient with GPP. However, these treatments aren’t always successful. GPP frequently recurs in Malaysia,” the Galen Centre noted.[4]
Some dermatologists may also suggest medications like non-steroidal anti-inflammatory drugs (NSAIDs), muscle relaxants, or antispasmodic treatments to help treat symptoms of psoriatic arthritis. Physical therapy is another option to strengthen the muscles and improve mobility and range of motion. In some cases, anaesthetic or corticosteroid injections may be used to treat pain.[4]
Most Countries Lack Medications Specifically For GPP
The majority of countries, including Malaysia, do not have any medications that have been specifically approved to treat GPP, nor are there any national or regional advice for managing the rare condition.[4]
Some of the current treatments for GPP include acutretin, methotrexate, cyclosporin, and biologics (Choon, 2022).[2]
The Galen Centre noted that Malaysia is currently carrying out several studies and clinical trials to treat GPP.[4]
“In a few years, patients with GPP may be given a highly effective targeted therapy if the impressive results of earlier studies are repeated.”[4]
Recommendations
The Galen Centre made five recommendations in its White Paper, including to implement a National Psoriasis Programme; to improve access to earlier interventions, therapies, and care; and to empower people living with GPP to speak up.[4]
The think tank also called for enhanced benefits under the Employees Provident Fund (EPF) and Social Security Organisation (Socso), as well as insurance coverage, to classify GPP as a critical illness, a “bare minimum”.[4]
While an outstanding example of a disease registry, there is room for improvement for the Malaysian Psoriasis Registry, said the Galen Centre.
Recommendations emerging from the expert meeting organised by the Galen Centre included creating a GPP support subgroup, ensuring sufficient GPP patient education material, and facilitating roles of nurse educators who act as a bridge between patients and doctors.[3]
Experts also suggested educating primary care and other physicians, increasing chances of patient involvement in clinical trials and to regularly update patients on the latest treatments available, and using a budget impact analysis to convince policymakers in efforts to register or subsidise specific drugs.[3]
The claim limit for insurance policies should also be increased, experts said, noting that skin conditions are not “entirely aesthetics related”.[3]
REFERENCES
- Choon, S.E., Lai, N.M., Mohammad, N.A., Nanu, N.M., Tey K.E., Chew, S.F. (2014). Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 53(6):676-84. doi: 10.1111/ijd.12070.
- Choon, S.E. (2022). The Future of Treatment in Generalized Pustular Psoriasis. International Psoriasis Council. [Link].
- Galen Centre for Health and Social Policy (2023). Challenges in Medicine – Living With Generalised Pustular Psoriasis In Malaysia: Challenges and Opportunities [Event Report].
- Galen Centre for Health and Social Policy (2022). Understanding unmet needs in treatment and care of generalized pustular psoriasis in Malaysia [White Paper].
- Gooderham, M.J., Van Voorhees, A.S., & Lebwohl, M.G. (2019) An update on generalized pustular psoriasis, Expert Review of Clinical Immunology, 15(9). DOI: 10.1080/1744666X.2019.1648209
- Navarini, A.A., Burden, A.D., Capon, F., et al. (2017). European consensus statement on phenotypes of pustular psoriasis. ERASPEN Network. J Eur Acad Dermatol Venereol, 31.
- Neuhauser, R., Eyerich, K., Boehner, A. (2020). Generalized pustular psoriasis—dawn of a new era in targeted immunotherapy. Exp Dermatol, 29.
- Sampogna, F., Tabolli, S., Söderfeldt, B., et al. (2006). Measuring quality of life of patients with different clinical types of psoriasis using the SF-36. Br J Dermatol, 154.
- Sobell, J.M., Gao, R., Golembesky, A.K., et al. (2021). Healthcare Resource Utilization and Baseline Characteristics of Patients With Generalized Pustular Psoriasis: Real-World Results From a Large US Database of Multiple Commercial Medical Insurers. Journal of Psoriasis and Psoriatic Arthritis, 6(3). Doi:10.1177/24755303211021779
- Umezawa, Y., Ozawa, A., Kawasima, T., et al. (2003). Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity. Arch Dermatol Res, 295.
- Zelickson, B.D., Muller, S.A. (1991). Generalized pustular psoriasis: A review of 63 cases. Arch Dermatol, 127.
