The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study

BackgroundSince the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers.ObjectiveTo analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center.MethodsWe reviewed the medical files of 740 patients treated between 1998 and 2022. Treatment results were collected, efficacy was assessed by a grading scale and acute adverse effects were registered.ResultsWe treated patients with 26 different diseases. We registered marked improvement (MI) or complete remission (CR) in 42.8% of patients with morphea, 50% with Urticaria Pigmentosa, 40.7% with Granuloma annulare and 85.7% with skin sarcoidosis. Good results were obtained also in the treatment of chronic Graft Versus Host Disease (GVHD), Eosinophilic Fasciitis, Sclero-atrophic Lichen, skin manifestations of systemic lupus erythematosus and psoriasis of HIV+ patients. Systemic Sclerosis, Romberg’s Syndrome, Bushke’s Scleredema, Nephrogenic Fibrosing Dermopathy, REM Syndrome, Follicular Mucinosis, Pretibial Myxedema, Scleromyxedema, pemphigus foliaceus, chronic cutaneous lupus erythematosus, erythroderma of Netherton Syndrome and Necrobiosis Lipoidica were no or poorly responsive. In clinical indications where UVA1 was used as a second line phototherapy after narrow-band (NB)-UVB, we saw good MI or CR rates in Mycosis Fungoides (57% of patients), Atopic Dermatitis (33.9%), Pitiryasis Lichenoides chronica (50%), Pityriasis Lichenoides et varioliformis acute (75%) and Lymphomatod Papulosis (62.5%). Short-term adverse events were uncommon and mild.ConclusionOver the past decade, the annual number of treated patients has progressively declined for several reasons. Firstly, UVA1 phototherapy has taken a backseat to the cheaper and more practical NB-UVB phototherapy, which has proven effective for common indications. Secondly, the emergence of new, safe, and effective drugs for conditions such as atopic dermatitis, GVHD, and connective tissue disorders. Finally, our research has shown that UVA1 therapy is often ineffective or minimally effective for some rare diseases, contrary to previous case reports and small case series. Nonetheless, UVA1 continues to be a valuable treatment option for patients with specific skin disorders

Proactive Management with Cal/BD Foam in Patients with Plaque Psoriasis prolongs Time with improved health-related Quality of Life when compared with reactive Management

Hintergrund: Die Phase-III-Studie PSO-LONG (NCT02899962) zeigte, dass ein proaktives Management (PM) mit Cal/BD-Schaum (Calcipotriol 0,005 %/Betamethason Dipropionat 0,064 %) gegenüber einem reaktiven (RM) über bis zu 52 Wochen bei Erwachsenen mit Psoriasis zu einer überlegenen Wirksamkeit führt. [1] Der von Patienten angegebene DLQI [2] bewertet deren Wahrnehmung der Psoriasis hinsichtlich der gesundheitsbezogenen Lebensqualität (HRQoL). Diese Post-hoc-Analyse von PSO-LONG untersucht, ob das initiale DLQI-Ansprechen nach Cal/BD-Schaumbehandlung in der Open-Label-Phase besser durch anschließendes PM oder RM gehalten werden konnte. Ziele: 1) Hervorheben, dass eine 1x tgl. offene Behandlung mit Cal/BD-Schaum über 4 Wo. die HRQoL von Plaque-Psoriasis-Patienten verbessert 2) Zeigen, dass ein PM mit Cal/BD-Schaum über bis zu 52 Wo. die anfängliche HRQoL-Response, die nach einer 4-wöch. offenen Cal/BD-Schaum-Behandlung erreicht wurde, im Vergleich zum RM bei Plaque-Psoriasis-Patienten signifikant verlängert Methoden: PSO-LONG beinhaltete eine initiale 4-wöchige Open-Label-Phase (OLP) (1x tgl. Cal/BD-Schaum) und eine 52-wöchige doppelblinde Erhaltungsphase (EP), in der Patienten randomisiert zweimal wöchentlich Cal/BD- oder Vehikel-Schaum (PM bzw. RM) anwendeten. Bei Rezidiven (Physician’s Global Assessment [PGA] ≥2) wurde über 4 Wo. 1x tgl. Cal/BD-Schaum gegeben. Der Anteil der Patienten mit einem DLQI = 0/1 nach der OLP wurde während der EP weiter beobachtet, um festzustellen, wie lange anfänglich erzielte Ergebnisse anhielten. Pro Gruppe wurden Kaplan-Meier-Kurven und Hazard Ratios (HR) für die Zeit mit Ansprechen ausgewertet. Ergebnisse: Die Analyse umfasste 521 Patienten mit überwiegend PGA-moderater Psoriasis (85,2 %). Während der OLP erreichten 49 % der Patienten einen DLQI = 0/1. Während der EP war das RM versus PM mit fast doppelt so hohem Risiko assoziiert, den DLQI = 0/1 zu verlieren (HR: 1,92; p\u3c0,001), und die mediane Zeit bis zum Ansprechverlust war fast 3,5x kürzer (57 bzw. 197 Tage). Fazit: Bei einer Untergruppe von Patienten, die nach initialer Behandlung mit Cal/BD-Sprühschaum ein DLQI = 0/1 HRQoL-Ansprechen erreichten, verlängerte das anschließende PM mit Cal/BD-Schaum die Zeit mit DLQI-Ansprechen signifikant gegenüber RM

Expanding the clinical and mutational spectrum of B4GALT7-spondylodysplastic Ehlers-Danlos syndrome

Spondylodysplastic EDS (spEDS) is a rare connective tissue disorder that groups the phenotypes caused by biallelic B4GALT7, B3GALT6, and SLC39A13 mutations. In the 2017 EDS nosology, minimal criteria (general and gene-specific) for a clinical suspicion of spEDS have been proposed, but molecular analysis is required to reach a definite diagnosis. The majority of spEDS patients presented with short stature, skin hyperextensibility, facial dysmorphisms, peculiar radiological findings, muscle hypotonia and joint laxity and/or its complications. To date only 7 patients with β4GALT7-deficiency (spEDS-B4GALT7) have been described and their clinical data suggested that, in addition to short stature and muscle hypotonia, radioulnar synostosis, hypermetropia, and delayed cognitive development might be a hallmark of this specific type of spEDS. Additional 22 patients affected with an overlapping phenotype, i.e., Larsen of Reunion Island syndrome, all carrying a homozygous B4GALT7 mutation, are also recognized. Herein, we report on a 30-year-old Moroccan woman who fitted the minimal criteria to suspect spEDS, but lacked radioulnar synostosis and intellectual disability and presented with neurosensorial hearing loss and limb edema of lymphatic origin. Sanger sequencing of B4GALT7 was performed since the evaluation of the spEDS gene-specific minor criteria suggested this specific subtype. Mutational screening revealed the homozygous c.829G>T, p.Glu277* pathogenetic variant leading to aberrant splicing. Our findings expand both the clinical and mutational spectrum of this ultrarare connective tissue disorder. The comparison of the patient's features with those of the other spEDS and Larsen of Reunion Island syndrome patients reported up to now offers future perspectives for spEDS nosology and clinical research in this field

AtopyReg®, the Prospective Italian Patient Registry for Moderate‐to‐Severe Atopic Dermatitis in Adults: Baseline Demographics, Disease Characteristics, Comorbidities, and Treatment History

Background and Objective AtopyReg® is a multicenter, prospective, observational, non-profit cohort study on moderate-tosevere atopic dermatitis in adults promoted in 2018 by the Italian Society of Dermatology and Venereology (SIDeMaST). We aimed to describe baseline demographics, disease characteristics, comorbidities, and therapeutic data of adult patients affected by moderate-to-severe atopic dermatitis. Methods Patients were selected based on the following inclusion criteria: age ≥ 18 years; Eczema Area and Severity Index score ≥ 16 or localization in visible or sensitive areas (face, neck, hands, or genitalia), or a Numeric Rating Scale itch score ≥ 7 or a Numeric Rating Scale sleep loss score ≥ 7, or a Dermatology Life Quality Index score ≥ 10. Demographic and clinical data at baseline were recorded and analyzed. Results A total of 1170 patients (male 51.1%; mean age: 44.7 years; range 18–90 years) were enrolled by 12 Italian Dermatology Units between January 2019 and November 2022. Skin lesions were eczematous in 83.2% of patients, the most involved site were the flexures (53.9%), face (50.9%), and neck (48.0%). Mean Eczema Area and Severity Index score was 22.3, mean Dermatology Life Quality Index value was 17.6, mean Patient Oriented Eczema Measure score was 13.1, and mean Numeric Rating Scale itch and sleep loss scores were 7.6 and 5.9, respectively. Previous systemic therapies were corticosteroids in 77.7% of patients, antihistamines in 50.3% of patients, and cyclosporine A in 42.6% of patients. Conclusions This baseline data analysis deriving from AtopyReg ® provides real-life evidence on patients with moderateto- severe atopic dermatitis in Italy confirming the high burden of atopic dermatitis with a significant impact on patients’ quality of life

P54 Incidence of skin cancer in systemic lupus erythematosus compared with systemic sclerosis and general population

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Survival, efficacy and safety of tralokinumab after 32 and 52 weeks of treatment for moderate-to-severe atopic dermatitis in adults: A multicentre real-world study

, efficacy and safety of tralokinumab after 32 and 52weeks of treatment for moderate-to-severe atopic dermatitis in adults: A multicentre real-world stud

Long-term topical management of psoriasis: the road ahead

AbstractTopical therapies have been available for the treatment of psoriasis for several decades. Despite this and the availability of several types of topicals, with varying potency, and numerous ..

Echocardiographic evaluation of diastolic dysfunction in young and healthy patients with psoriasis: A case-control study

Psoriasis is a systemic inflammatory disease with a great prevalence in general population. The inappropriate activation of the cellular immune system has been hypothesized to be an independent cardiovascular risk factor, given the higher incidence of cardiovascular disorders in psoriatic patients. Echocardiographic abnormalities have been demonstrated too: the aim of our study was to evaluate the presence of preclinical cardiac dysfunction in a cohort of psoriatic patients without cardiovascular risk factors. We enrolled 52 patients with the diagnosis of chronic plaque psoriasis, compared with a control group not affected by any relevant systemic diseases and inflammatory disorders. In all patients and control group, echocardiographic conventional and tissue Doppler (TDI) studies were conducted. The analysis of echocardiographic parameters revealed normal dimension, mass and systolic function of the left ventricle. Left ventricular diastolic dysfunction was found in 36.5% patients in the psoriasis group versus 0% in control group, and significant reduction of the E/A ratio was found also for the right ventricle. A significant increase of mitral regurgitation has been found in psoriatic patients (p=0.005). The early recognition of cardiovascular pre-clinic disease in psoriatic patients may guide a strict follow up and an early treatment, potentially improving cardiovascular prognosis