JOYCE Choo hesitates to shake my hand, just for a second. It's understandable given the way people usually react to her.
Since her schooldays, Choo has had to put up with stares and curious looks from strangers thanks to her skin condition.
She has always tried her best to hide her condition, wearing long-sleeved pants and shirts in an attempt to cover her hands and legs, but still, people stare or point at her face and it has impacted her self-esteem greatly.
Choo suffers from generalised pustular psoriasis (GPP), a rare and severe form of psoriasis that can be potentially life-threatening. GPP can appear suddenly with many painful, pus-filled blisters all over the body or more gradually with ring-shaped patches. A GPP flare can last from weeks to months, and it often recurs.
Choo, a wife and mum, has been grappling with the condition since her schooldays, resulting in frequent hospital admissions and missed schooling days, and even the inability to pursue higher education. But the biggest impact has been to her self-worth and her ability to socialise.
"People used to stare at me like I was some kind of alien."
At work, she used to agonise over interacting with colleagues, always conscious of how she looked, and anxious about whether her colleagues were comfortable sharing office space with her or going out for lunch together.
Choo says so many of the everyday things that people take for granted is a huge challenge for someone with GPP because people's perceptions of someone is always affected by how they look.
"I have never worn short skirts or sleeveless tops, even as a teenager, because I was always worried about people staring at my skin."
The condition made her shy and withdrawn, always conscious of her appearance.
She experienced GPP flares several times a year which affected every aspect of her life.
In 2016, having exhausted other treatment options for GPP she was put on biologics, which helped her flares to subside significantly and improved her quality of life.
GPP can be life-threatening if left untreated, due to potential complications such as infections or organ failure.
Treatments often include systemic medications aimed at controlling inflammation and preventing flare-ups. With treatment, the pustules may gradually disappear, leaving behind scales that lead to hyperpigmentation.
According to the Johor cohort study published in 2023 in the 'British Journal of Dermatology', between 2010 and 2020, 230 people were estimated to be living with GPP in a population of 1.1 million, which amounts to 198 per million individuals, or approximately 0.02 per cent of the country's population.
The condition appears to be more prevalent among females and is particularly high among the Chinese compared with Indians and Malays.
Choo says it can be a challenge trying to explain her condition to people and often they respond with all sorts of suggestions or home remedies, not realising just how serious GPP really is.
"So much of my time and energy has been taken up in coping with my condition. Few people realise just how big an impact it has on daily life."
Quite often, those with GPP are misdiagnosed, adds Raja Permaisuri Bainun Ipoh Hospital head of department and consultant dermatologist Dr Tang Jyh Jong. Patients may be initially told they have a bacterial infection and sent home with antibiotics.
"Early and timely intervention is crucial as is access to effective treatment."
While GPP may be a rare condition, it is potentially life-threatening and infinitely challenging for the patient, he points out.
In general, a combination of genetic and environmental risk factors, may trigger or exacerbate GPP and these include viral or bacterial infections such as streptococcal, trichophyton rubrum, cytomegalovirus, Epstein-Barr virus and the varicella-zoster virus.
There is also substantial evidence that withdrawal of systemic corticosteroid therapy can precipitate GPP, and topical therapy with strong corticosteroids has also been implicated.
Many individuals also report deterioration at times of psychological stress, while pregnancy has been recognised as a risk factor too. This form of GPP usually occurs during the third trimester and may recur during subsequent pregnancies.
NOT JUST PSORIASIS
Generalised pustular psoriasis (GPP) is distinct from plaque psoriasis (the most common form of psoriasis) in a few ways. GPP is a neutrophilic autoinflammatory disease, a condition characterised by a buildup of neutrophils (a type of white blood cell) in the skin, whereas plaque psoriasis is considered an autoimmune disease where the immune system attacks and damages the body's own tissues instead of protecting it.
They are also different in appearance and severity. While plaque psoriasis presents with raised, red patches covered in silvery scales, GPP is characterised by widespread pustules filled with non-infectious pus on inflamed, red skin.
GPP can cause systemic symptoms such as fever, chills and fatigue, making it a medical emergency requiring more aggressive treatment in severe cases. In contrast, plaque psoriasis is typically a chronic condition with slower progression and fewer acute, life-threatening symptoms.
Newer findings and medical advancements have now made it possible to develop targeted therapies for the treatment of GPP, in particular, medications that block proteins that cause inflammation.