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.

Tildrakizumab for the treatment of moderate-to-severe psoriasis: a 52-week, real-world Portuguese multicentric study.

Background: Real-world evidence plays a pivotal role in validating the efficacy of biologic drugs beyond the controlled environment of randomized trials. This study aimed to evaluate the effectiveness of tildrakizumab in treating moderate-to-severe psoriasis within a real-world setting over a 52-week period in Portugal. Methods: This multicentric, prospective, observational study included adult patients with moderate-to-severe psoriasis. All participants received tildrakizumab 100 mg at weeks 0 and 4, followed by a maintenance dose every 12 weeks, and were monitored for 52 weeks. Primary endpoints were determined based on Psoriasis Area and Severity Index (PASI) assessments at baseline, 16 (±2) weeks, 28 (±2) weeks and 52 (±2) weeks. Results: A total of 54 patients were enrolled in the study (56% men, mean age of 50.3 ± 14.4 years). Half of the sample (n=27) had no prior experience with biologic treatments. About 74% of patients (n=40) presented at least one comorbidity during the study, with psoriatic arthritis being the most prevalent (29.6%). By week 52, there was a significant decrease in the mean PASI from 17.8±10.3 at baseline to 1.3±1.9 (p<0.001), indicating an overall improvement of 93%. By week 52, more than 85% of patients attained PASI ≤5, more than 80% reached PASI ≤3, and nearly 60% achieved PASI ≤1. Infections were observed in 9.3% of patients, and one patient required hospitalization (1.9%). The cumulative proportion of patients continuing treatment at 52 weeks was 88.9%. Conclusions: This study demonstrates that tildrakizumab is an effective and safe agent for the treatment of moderate-to-severe psoriasis in a diverse, real-world setting.

Can Collagen Membrane on Bone Graft Interfere with Light Transmission and Influence Tissue Neoformation During Photobiomodulation? A Preliminary Study.

Objective: This study qualitatively and quantitatively evaluated the transmission of light through a collagen membrane and the consequent local bone formation in a critical bone defect in vitro and in an animal model. Background: Currently, bone substitutes and collagen membranes are used to promote new bone formation; however, when associated with photobiomodulation, biomaterials can act as a barrier, hindering the passage of light radiation to the area to be treated. Methods: Light transmittance was evaluated in vitro with a power meter and a 100 mW, 808 nm laser source with and without membrane. Twenty-four male rats received a critical surgical defect of 5 mm in diameter in the calvarial bone, subsequently a biomaterial (Bio-Oss; Geistlich®, Switzerland) was applied, and the animals were divided into the following three groups: G1-collagen membrane and no irradiation; G2-collagen membrane and photobiomodulation (irradiation with 4 J of 808 nm); and G3-photobiomodulation (4 J) followed by a collagen membrane. Histomophometric analyses were performed at 7 and 14 days after euthanasia. Results: The membrane reduced the light transmittance (808 nm) by an average of 78%. Histomophometric analyses showed significant differences in new blood vessels on day 7 and bone neoformation on day 14. Irradiation without membrane interposition resulted in a 15% more neoformed bone compared with the control (G1), and 6.5% more bone compared with irradiation over the membrane (G2). Conclusions: The collagen membrane interferes with light penetration during photobiomodulation, decreases light dosimetry on the wound area, and interferes with bone neoformation.

Advances, Challenges, and Future Perspectives of Microwave Reflectometry for Plasma Position and Shape Control on Future Nuclear Fusion Devices.

Providing energy from fusion and finding ways to scale up the fusion process to commercial proportions in an efficient, economical, and environmentally benign way is one of the grand challenges for engineering. Controlling the burning plasma in real-time is one of the critical issues that need to be addressed. Plasma Position Reflectometry (PPR) is expected to have an important role in next-generation fusion machines, such as DEMO, as a diagnostic to monitor the position and shape of the plasma continuously, complementing magnetic diagnostics. The reflectometry diagnostic uses radar science methods in the microwave and millimetre wave frequency ranges and is envisaged to measure the radial edge density profile at several poloidal angles providing data for the feedback control of the plasma position and shape. While significant steps have already been given to accomplish that goal, with proof of concept tested first in ASDEX-Upgrade and afterward in COMPASS, important, ground-breaking work is still ongoing. The Divertor Test Tokamak (DTT) facility presents itself as the appropriate future fusion device to implement, develop, and test a PPR system, thus contributing to building a knowledge database in plasma position reflectometry required for its application in DEMO. At DEMO, the PPR diagnostic's in-vessel antennas and waveguides, as well as the magnetic diagnostics, may be exposed to neutron irradiation fluences 5 to 50 times greater than those experienced by ITER. In the event of failure of either the magnetic or microwave diagnostics, the equilibrium control of the DEMO plasma may be jeopardized. It is, therefore, imperative to ensure that these systems are designed in such a way that they can be replaced if necessary. To perform reflectometry measurements at the 16 envisaged poloidal locations in DEMO, plasma-facing antennas and waveguides are needed to route the microwaves between the plasma through the DEMO upper ports (UPs) to the diagnostic hall. The main integration approach for this diagnostic is to incorporate these groups of antennas and waveguides into a diagnostics slim cassette (DSC), which is a dedicated complete poloidal segment specifically designed to be integrated with the water-cooled lithium lead (WCLL) breeding blanket system. This contribution presents the multiple engineering and physics challenges addressed while designing reflectometry diagnostics using radio science techniques. Namely, short-range dedicated radars for plasma position and shape control in future fusion experiments, the advances enabled by the designs for ITER and DEMO, and the future perspectives. One key development is in electronics, aiming at an advanced compact coherent fast frequency sweeping RF back-end [23-100 GHz in few µs] that is being developed at IPFN-IST using commercial Monolithic Microwave Integrated Circuits (MMIC). The compactness of this back-end design is crucial for the successful integration of many measurement channels in the reduced space available in future fusion machines. Prototype tests of these devices are foreseen to be performed in current nuclear fusion machines.

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.

Comparative effectiveness of purpuragenic 595 nm pulsed dye laser versus sequential emission of 595 nm pulsed dye laser and 1,064 nm Nd:YAG laser: a double-blind randomized controlled study.

INTRODUCTION: Erythematotelangiectatic rosacea is a common condition in Caucasians. The most frequently used lasers to treat this condition are pulsed dye laser (PDL) and neodymium:yttrium-aluminum-garnet laser (Nd:YAG). This study compares the treatment efficacy of purpuragenic PDL with that of sequential emission of 595 nm PDL and 1,064 nm Nd:YAG (multiplexed PDL/Nd:YAG). METHODS: We performed a prospective, randomized, and controlled split-face study. Both cheeks were treated, with side randomization to receive treatment with PDL or multiplexed PDL/Nd:YAG. Efficacy was evaluated by spectrophotometric measurement, visual photograph evaluation, the Dermatology Quality of Life Index questionnaire, and a post-treatment questionnaire. RESULTS: Twenty-seven patients completed the study. Treatment was associated with a statistically significant improvement in quality of life (p < 0.001). PDL and multiplexed PDL/Nd:YAG modalities significantly reduced the erythema index (EI; p < 0.05). When comparing the degree of EI reduction, no differences were observed between the two treatment modalities. PDL was associated with a higher degree of pain and a higher percentage of purpura. Multiplexed PDL/Nd:YAG modality was associated with fewer side effects and greater global satisfaction, and 96.3% of the patients would recommend this treatment to a friend. CONCLUSIONS: Both laser modalities are efficacious in the treatment of erythematotelangiectatic rosacea. The multiplexed PDL/Nd:YAG modality was preferred by the patients.

Near-total lower lip reconstruction: combined Karapandzic and Bernard-Burrow-Webster flap.

Lip carcinoma is a common cancer of the head and neck region. It more often affects the lower lip (> 90%), and squamous cell carcinoma represents 95% of the cases. The authors present a reconstruction of the lower lip combining two classical flaps: the Karapandzic and Bernard-Burrow-Webster flap. The surgical technique is discussed.

Evaluation of hygiene habits: cross-sectional study.

Introduction: It is well known that adequate hygiene is important for health. Even though this topic has drawn the attention of the media, little or no scientific investigation has been done. Methods: We performed a comparative questionnaire-based cross-sectional study in three groups: patients attending a dermatology outpatient clinic, patients attending an internal medicine consultation, and community members. Results: We analyzed a total of 446 questionnaires (249 from dermatology patients, 98 from internal medicine patients, and 99 from the community group). The three groups did not differ statistically in sex and age (p=0.070). The patients from the dermatology department had a higher education level. The number of weekly baths did not differ among the three groups (p=0.417). Hair hygiene did not differ between the three groups. The dermatology and internal medicine groups washed their hands more frequently than the community group (p=0.028). Conclusions: Comparing our results to the limited data available, we find that the population surveyed has better hygiene habits than those previously reported. We believe that hygiene habits should be discussed during office visits.

Gaps and barriers to tuberculosis screening among anti-tumor necrosis factor prescribers.

Delegates from the Tuberculosis Committee of the Portuguese Pulmonology Society, the Portuguese Rheumatology Society, the Portuguese Dermatology and Venereology Society and the Portuguese Gastroenterology Society, have revised and updated, in 2012, their guidelines for the diagnosis and treatment of latent tuberculosis infection and active tuberculosis in patients that are candidates for therapy with biologic drugs. In order to identify perceived barriers to tuberculosis screening among patients candidate to anti-TNF treatment, we performed a cross-sectional survey including rheumatologists, gastroenterologists and dermatologists who prescribed anti-TNF agents, identified by the respective Scientific Societies, throughout Portugal. Ninety-five physicians (85 specialist and 10 trainees with more than 3 years of practice) participated in the survey, including 42 rheumatologists (response rate 28%), 32 dermatologists (12% response) and 21 gastroenterologists (4% response). No information was collected on non-respondents. This study showed that most of the participants were aware of tuberculosis risk and that they screened patients for tuberculosis following guidelines.

Inverse psoriasis treated with ustekinumab.

Inverse psoriasis is characterised by the involvement of flexural skin folds. This form of psoriasis has distinct clinical and therapeutic features. This report refers to the case of a 48-year-old Caucasian man who was observed in our department, with a clinically and biopsy proven diagnosis of inverse psoriasis. For 2 years, the patient was treated with different combinations of corticosteroids, vitamin D analogues and methotrexate, with no satisfactory response. Given the lack of a clinical response and comorbidities, latent tuberculosis was excluded, and we started treatment with ustekinumab. We chose this biological agent because the patient was a long-distance truck driver and refused the possibility of autoinjections. The patient underwent three ustekinumab injections, which resulted in significant improvement of pruritus, erythaematous lesions and quality of life.

Treatment of thalidomide resistant pyoderma gangrenosum with etenercept.

Tumor necrosis factor alfa (TNF-α) is a cytokine with a central role in inflammation. Its blockade can be achieved by using specific inhibitors like etanercept. At present, etanercept is approved for rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, juvenile idiopathic arthritis and psoriasis treatment. However, it has been used with promising results in other inflammatory dermatoses. A case is presented of a 54-year-old man diagnosed with pyoderma gangrenosum. He completed treatment with oral prednisolone with favorable outcome, but with recurrence after glucocorticoid therapy withdrawal. Thalidomide was introduced, but after complaints of glove and sock paresthesia and hypoesthesia, the drug was discontinued. Taking into account the favorable outcome with thalidomide and the necessity of drug withdrawal, it was decided to introduce therapy acting on the same step in the inflammation cascade. In this regard, etanercept was initiated. The aim of this article is to present an alternative therapy for refractory pyoderma gangrenosum, along with a literature review.

Primary cutaneous blastoid mantle cell lymphoma-case report.

Mantle cell lymphoma (MCL) commonly involves extranodal sites, usually as a manifestation of disseminated disease. In rare cases, MCLs may arise as a primary tumor in the skin. Blastoid mantle cell lymphoma (BV-MCL) is a rare variant and has a more aggressive clinical course. The phenotype of BV-MCL is characterized as CD20+, CD5+, cyclin D1+, CD23-, and CD10-. Interphase fluorescence in situ hybridization shows a characteristic t(11;14) fusion pattern. We report a case of a BV-MCL arising in skin as primary cutaneous MCL with the characteristic immunophenotype and translocation.

Description and management of cutaneous side effects during erlotinib and cetuximab treatment in lung and colorectal cancer patients: a prospective and descriptive study of 19 patients.

Erlotinib and cetuximab are human epidermal growth factor receptor inhibitors (EGFRI) that are approved in monotherapy for the treatment of patients with locally advanced or metastatic non-small cell lung cancer after failure of at least one prior chemotherapy regimen. Papulopustular eruptions are the most frequent adverse effect, their occurrence being associated with increased survival in some studies. We describe 19 patients who presented with a rash located mainly to the face and trunk, without presence of comedones, shortly after initiation of EGFRI therapy. We present our algorithm to manage these patients and their respective responses. We also report other therapeutic options and cutaneous alterations that may be seen.