Clinical course and disease burden of patients with generalized pustular psoriasis in Portugal: a multicenter retrospective cohort study.

OBJECTIVES: Generalized pustular psoriasis (GPP) is a rare, life-threatening skin inflammatory disorder. This study aimed to describe the disease course, treatment strategies, and healthcare utilization among patients with GPP in Portugal. METHODS: This multicentric, observational, retrospective study included consecutive adult patients with GPP undergoing a dermatology evaluation in different reporting institutions by experienced dermatologists between 2002 and 2023. RESULTS: A total of 59 patients were assessed. Most of the cohort had a previous history of plaque psoriasis (71%) and 83% presented at least one comorbidity. At the initial encounter, 64% of the cohort needed hospitalization. Systemic involvement was common, including fever (37%), and elevated white blood cell count and erythrocyte sedimentation rate/C-reactive protein (49%). Nearly, 73% of patients initiated systemic drugs, and 70% had to discontinue the first treatment. During the study, 98% of patients experienced at least one flare. At the last visit, 3.4% of patients had died, and 71.2% exhibited signs of active disease despite undergoing treatment. CONCLUSIONS: Our study demonstrates that GPP is a chronic, debilitating condition associated with systemic involvement, frequent flares, and hospitalizations, despite receiving multiple systemic treatments. Improved disease awareness and new treatments are needed to improve patient care and decrease the burden of the disease.

Epidemiology of Psoriasis in Portugal: A Population-Based Study.

INTRODUCTION: Psoriasis is a common, chronic, and inflammatory skin disorder with a high personal, social and economic burden and important implications for healthcare systems. The aim of this study was to provide an epidemiological characterization of individuals with psoriasis in Portugal. MATERIAL AND METHODS: A large observational, cross-sectional, nationwide, population-based survey study developed by the Portuguese Psoriasis Group of the Portuguese Society of Dermatology and Venereology (GPP-SPDV). A structured questionnaire was designed and applied by experienced interviewers to a random, representative sample of Portuguese individuals with psoriasis and/or psoriatic arthritis. Patients were considered to have psoriasis if they replied positively to one of the following questions: "Does any physician have ever diagnosed you with psoriasis?" or "Do you have a skin disorder characterized by scaling, reddish skin lesions located in the elbows/knees/scalp?". RESULTS: A total of 6381 individuals were interviewed, of which 283 met the criteria for psoriasis, corresponding to a prevalence rate of 4.4% (95% CI 3.95 - 4.98). Out of the participants that met psoriasis criteria, 24% had suggestive signs/symptoms but did not have a clinical diagnosis established and were not being monitored by a physician. Although more than 70% of participants had active disease (scaling, erythema, or pruritus) and one third had joint symptoms, only 12% were on systemic treatment. Fifty percent of participants with psoriasis (n = 139) had relevant comorbidities (most frequently depression/anxiety and cardiometabolic diseases). Sixteen percent of participants with psoriasis (n = 46) reported that psoriasis interfered with their daily activities (median impact of 5 in a 0 - 10 scale) and 12% mentioned the disease had an impact in their sexual life (median impact of 5 in a 0 - 10 scale). CONCLUSION: The results of this study suggest that the prevalence rate of psoriasis is likely to be high in Portugal, and several gaps exist at different levels of healthcare delivery to these patients, from diagnosis to treatment. This study provides important data for the future planning of interventions targeting the improvement of psoriasis care in Portugal.

Dermatologists' attitude towards psoriasis treatment during the COVID-19 pandemic.

BACKGROUND: The COVID-19 pandemic introduced new challenges in several dimensions in healthcare services. Herein, we describe the real-life strategies and therapeutic options adopted by dermatologists regarding their patients with psoriasis being treated with or with an indication for systemic therapy during the first COVID-19 lockdown period in Portugal. METHODS: The study involves a web-based survey on the clinical management of systemic therapy for psoriasis during the COVID-19 pandemic administered to Portuguese dermatologists. The survey consisted of 55 questions (4 open-ended questions; 51 closed-ended questions), grouped into 6 sections. RESULTS: A total of 60 dermatologists voluntarily participated in this survey. Nearly 63% of the participants opted for suspending biologics during the COVID-19 lockdown period and 23.3% increased the time between drug administrations. Eighty percent of the participants agreed that biologics did not change the probability of acquiring COVID-19 and 58.4% believed that these drugs decreased or did not change the severity of the disease. Approximately one-third of the participants opted not to prescribe a biological agent in patients despite clinical indication over the duration of the pandemic. Nearly 25% of the participants opted for suspending traditional immunosuppressant administration. Virtual appointments were an option for 93.3% of the participants. CONCLUSION: The COVID-19 pandemic has significantly affected the management of patients with psoriasis being treated with or with an indication for systemic therapy. Some of the decisions made during the first lockdown period were contrary to what we know today. These decisions might have had a significant impact on patients' quality of life and on future therapeutic success. An adequate interpretation and analysis of the available data will be extremely important to an insightful adaptation of the clinical practice in future confinement or restrictive scenarios.

Portuguese recommendations for the treatment of psoriasis with biologic therapy.

Psoriasis is a highly prevalent chronic, inflammatory multisystem disease with a considerable impact on patients' quality of life and the healthcare system. This report presents the recommendations developed by the Portuguese Psoriasis Group of the Portuguese Society of Dermatology and Venereology that address several clinical questions arising during the management and care of psoriasis with biologic therapy, based on the available evidence. The recommendations were generated following thorough evaluation of existing guidelines on the treatment of psoriasis, publications concerning new biologic treatments that have not yet been considered in existing guidelines, as well as expert-based recommendations. Considerations regarding the severity of psoriasis, indications for initiating biologic therapy, parameters to be considered in treatment choice (in particular, treatment goals), as well as recommendations for using and monitoring therapy and screening programmes are also included.

Erosive pustular dermatosis of the scalp. A chronic recalcitrant dermatosis developed upon CO2 laser treatment.

Erosive pustular dermatosis of the scalp (EPDS) is a rare, chronic inflammatory dermatosis that mostly affects elderly patients, who develop erosions, pustulation, crusting and scarring on the scalp. Its aetiology remains elusive, although the role of local trauma is being emphasized. Treatment is difficult, with several topical and systemic agents being reported to induce improvement. A 63-year-old Caucasian male had been suffering from persistent painful pustules, erosions and crusts on his scalp for 2 years. The onset of the lesions followed a CO2 laser vaporization procedure to treat multiple actinic keratoses. Different topical and systemic treatments had unsuccessfully been tried. A 4-month course of bid 0.1% tacrolimus ointment, along with strict external photoprotection, resulted in dramatic improvement, sustained after careful tapering of tacrolimus. This case is interesting in that the scalp eruption followed CO2 laser treatment. Other cases have been associated with cryosurgery, radiotherapy, surgery, and 5-FU. In fact, to our knowledge, ours is the fourth reported case of EPDS following CO2 laser treatment. Our case also strengthens previous observations as to the efficacy and safety of topical calcineurin inhibitors in this dermatosis. This is noteworthy bearing in mind the atrophic character of the skin in EPDS, which limits the usefulness of chronically administered topical steroids.