sunbeds and carcinogenesis the need for new regulations and restrictions in europe from the euromelanoma perspective

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Polymorphous light eruption and benign summer light eruption

BACKGROUND/PURPOSE: Polymorphous light eruption (PLE) heterogeneity has been postulated, but the existence of benign summer light eruption (BSLE) is controversial. We studied the prevalence of the clinical patterns, criteria distinguishing BSLE from PLE, and diagnostic usefulness of phototest. METHODS: Five Italian Photodermatology Centres recruited retrospectively 346 patients with typical clinical history and/or presentation of PLE. Age, gender, skin type, family history and presence of atopy were considered. UVA and UVB MEDs and provocative phototests with UVA and UVB were obtained with a standardized procedure. Photopatch tests were applied according to the IRCDG rules. ANA were assessed by indirect immunofluorescence. RESULTS: Four criteria (predominance of women, shorter latency, uninvolvement of the face and absence of relapse during summer) identified BSLE in only 6.1% of cases. All had positive phototests, mostly with UVA. Uninvolvement of face, short latency and no seasonal relapses identified 11.7% patients, mostly with positive phototests to UVA. Short latency and no seasonal relapses in women identified 11.2% patients. Uninvolvement of face and no seasonal relapses in women identified 8.1% of patients. Uninvolvement of face and short latency in women identified 17.6% of patients. CONCLUSION: Criteria diagnosed BSLE in only a minority of patients, who were positive at phototesting, mostly with UVA

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

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

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

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

Desmoplastic Melanoma: Report of 5 Cases

Background. The clinical presentation of desmoplastic melanoma is often challenging. We report the experience of the Melanoma Unit of Spedali Civili University Hospital of Brescia, Italy. Method. Study subjects were drawn from 1770 patients with histologica confirmed melanoma. Within this group, desmoplastic melanoma developed in 5 patients. For each diagnosed melanoma, histological characteristics, treatment, and outcomes were evaluated. Results. Of the 5 patients described in this study, 2 were males and 3 females. The average age was 62.4 years ranging from 56 to 68 years. Breslow thickness ranged from 2.1 to 12 mm with a mean thickness of 5.8 mm. Primary treatment of 5 patients included a wide local excision of their primary lesions. Conclusions. Desmoplastic melanoma is a rare neoplasm which clinically may mimic other tumours or cutaneous infiltrate of uncertain significance. The diagnosis is hiastopathological and radical resection is necessary