Quality of Life Severely Affected for Patients with GPP

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Untreated and misdiagnosed generalized pustular psoriasis (GPP) can severely impact patients’ quality of life, according to a new study.¹ The study features 4 cases from clinical practices that highlight the difficulties patients have in receiving accurate diagnoses, effective treatment, and regular follow-ups while emphasizing the physical and emotional burden the disease can cause.

Case 1

The first case involved a 14-year-old, white male. The patient first showed signs of an itchy rash, which began on his feet before worsening and spreading to the entire body. Further symptoms included the darkening, thickening, and dystrophy of nails. A skin biopsy later confirmed the diagnosis of GPP. The patient was treated with topical antifungals, doxycycline, and off-label secukinumab throughout this process, which did not necessarily target the underlying disease at times.

The psychological aspects were complex in this case, due to the comorbidity of autism. Personalized care in quiet times of the day surrounded by familiar team members helped address his medical and emotional needs. There were also some diagnosis challenges, since the patient was overall in good physical health and had no family history of GPP, which could have led to the lack of a prompt diagnosis.

Case 2

The second case involved a 55-year-old, white male. The patient had a 20-year history of plaque psoriasis and was a heavy smoker for 40 years. His lesions were mostly on the scalp but flares/remissions were episodic and were managed throughout his life. After receiving oral corticosteroids for an upper respiratory infection, a pustular rash developed on the scalp, hands, feet, and trunk. He also had joint pain, fever, and malaise and was frequently misdiagnosed at an urgent care center, leading to a clinical referral.

At the dermatology follow-up, this hyperkeratotic rash was worsened with erythroderma and pustules of the palms and soles, anonychia and dactylitis of multiple digits on the hands and feet, fingernail loss, and purulent drainage. The intense pain prevented the patient from being able to perform normal daily activities such as wearing shoes, gripping doorknobs, and closing his fist, and ultimately caused him to take a disability leave from work. GPP was suspected after a comprehensive physical examination and identification of risk factors. For treatment, the patient received an oral prednisolone taper, off-label guselkumab, and clobetasol ointment for itching skin. He was also placed on a smoking cessation program.

Case 3

The third case involved a 33-year-old, white female. Her comorbidities included obesity, hypertension, and a history of depression and anxiety, as well as family history of well-controlled plaque psoriasis. The patient received multiple topical and systemic treatments throughout her disease duration including methotrexate, acitretin, adalimumab, etanercept, cyclosporine, secukinumab, apremilast, guselkumab, and brodalumab. Other treatment methods included modified Goeckerman therapy, infliximab, and infliximab/methotrexate combination therapy, but most were met with little to no response.

Over the course of 7 years, the patient was hospitalized several times in which 20% to 40% of her body surface area was covered with painful pustules and was accompanied by erythroderma, malaise, pain, and fever. Since the patient was a single parent with previous mental health issues, this case in particular highlighted the physical exhaustion and emotional burden that can come with GPP. 

Case 4

The final case involved a 59-year-old, Hispanic male. This patient also had several comorbidities including asthma, hypertension, heart disease, benign prostatic hypertrophy, and back pain. He initially presented with mild plaque psoriasis on his hands, elbows, and scalp and was treated with several medications throughout his disease duration such as systemic certolizumab pegol, topical halobetasol, calcipotriene, ixekizumab, and secukinumab. As symptoms such as swelling, erythema, and burning occurred, certain medications were stopped and replaced with alternatives. as it was believed that the patient was having drug-induced reactions.

After irregular appointments and persistent pain, the patient was hospitalized after a physical examination revealed intense erythema and scaling of the skin, with a low-grade fever and chills. A diagnosis was made after a skin biopsy and off-label risankizumab was initiated but quick and accurate care is needed to monitor GPP flares.

The rarity and heterogeneity of GPP make it difficult to diagnose and treat. Spesolimab was approved in 2022 as a first-in-class, GPP-specific therapy. Before this, treatment options primarily consisted of off-label use of agents used to treat plaque psoriasis, which can be ineffective.² The investigators noted that psychological aspects should be heavily considered by health care providers when administering treatment

“The potential severity and consequences of untreated GPP necessitate a prompt diagnosis, initiation of effective treatment, and regular follow-up and monitoring of patients’ conditions,” the authors wrote. “When managing GPP, a multidisciplinary approach is recommended to ensure that patients receive appropriate social and emotional support and effective medical care.”

References

1. Christianson, E, Heim, J, Pansch, LA, et al. Burden of illness and unmet needs in generalized pustular psoriasis: report of four cases. Journal of Dermatology for Physician Assistants 18(2):p 16-21, Spring 2024. | DOI: 10.1097/jdpa.0000000000000017

2. Krueger J, Puig L, Thaçi D. Treatment Options and Goals for Patients with Generalized Pustular Psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):51-64. doi:10.1007/s40257-021-00658-9