Statistical Study and Prediction of Variability of Erythemal Ultraviolet Irradiance Solar Values in Valencia, Spain

[EN] The goal of this study was to statistically analyse the variability of global irradiance and ultraviolet erythemal (UVER) irradiance and their interrelationships with global and UVER irradiance, global clearness indices and ozone. A prediction of short-term UVER solar irradiance values was also obtained. Extreme values of UVER irradiance were included in the data set, as well as a time series of ultraviolet irradiance variability (UIV). The study period was from 2005 to 2014 and approximately 250,000 readings were taken at 5-min intervals. The effect of the clearness indices on global irradiance variability (GIV) and UIV was also recorded and bi-dimensional distributions were used to gather information on the two measured variables. With regard to daily GIV and UIV, it is also shown that for global clearness index (k(t)) values lower than 0.6 both global and UVER irradiance had greater variability and that UIV on cloud-free days (k(t) higher than 0.65) exceeds GIV. To study the dependence between UIV and GIV the (2) statistical method was used. It can be concluded that there is a 95% probability of a clear dependency between the variabilities. A connection between high k(t) (corresponding to cloudless days) and low variabilities was found in the analysis of bi-dimensional distributions. Extreme values of UVER irradiance were also analyzed and it was possible to calculate the probable future values of UVER irradiance by extrapolating the values of the adjustment curve obtained from the Gumbel distribution.The translation of this paper was funded by the Universitat Politècnica de València, Spain. The research was supported by the Generalitat Valenciana within the project PROMETEO II 058.Gurrea-Ysasi, G.; Blanca Giménez, V.; Perez, V.; Serrano, M.; Moreno, J. (2018). Statistical Study and Prediction of Variability of Erythemal Ultraviolet Irradiance Solar Values in Valencia, Spain. 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Facial Angiofibroma Severity Index (FASI): reliability assessment of a new tool developed to measure severity and responsiveness to therapy in tuberous sclerosis-associated facial angiofibroma

Background. Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Topical sirolimus has recently been suggested as a potential treatment for TSC-associated facial angiofibroma (FA). Aim. To validate a reproducible scale created for the assessment of clinical severity and treatment response in these patients. Methods. We developed a new tool, the Facial Angiofibroma Severity Index (FASI) to evaluate the grade of erythema and the size and extent of FAs. In total, 30 different photographs of patients with TSC were shown to 56 dermatologists at each evaluation. Three evaluations using the same photographs but in a different random order were performed 1 week apart. Test and retest reliability and interobserver reproducibility were determined. Results. There was good agreement between the investigators. Inter-rater reliability showed strong correlations (> 0.98; range 0.97–0.99) with inter-rater correlation coefficients (ICCs) for the FASI. The global estimated kappa coefficient for the degree of intra-rater agreement (test–retest) was 0.94 (range 0.91–0.97). Conclusions. The FASI is a valid and reliable tool for measuring the clinical severity of TSC-associated FAs, which can be applied in clinical practice to evaluate the response to treatment in these patients

Sustained clinical effectiveness and favorable safety profile of topical sirolimus for tuberous sclerosis – associated facial angiofibroma

Background Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Facial angiofibroma appears in up to 80% of patients and has a considerable psychological impact. Various invasive procedures have been used, although they show limited effectiveness and potential adverse effects. Objectives To evaluate the sustained clinical benefits and safety profile of topical sirolimus applied to treat facial angiofibromas. Methods This study was a non-blinded, uncontrolled case-series comprising 10 patients with TSC-associated facial angiofibroma that was treated with 0.4% sirolimus ointment 3 times a week for 9 months. Patients were clinically evaluated at baseline and at 6, 12, 24 and 36 weeks. Plasma levels of sirolimus were determined. Results A sustained improvement was observed in erythema and in the size and extension of the lesions as early as the first weeks of treatment. Sirolimus plasma levels remained below detection limits (0.3 ng ⁄ mL) in all cases. The formula was well-tolerated with no local or systemic adverse effects. Conclusions Topical sirolimus seems to be an effective and safe medical alternative to surgery or laser-based treatments in patients with TSC-associated facial angiofibromas

Hereditary congenital hypopigmented and hyperpigmented macules (Westerhof syndrome) in two siblings

S IR, In 1978 Westerhof et al. reported several members of a family with multiple hereditary congenital hypopigmented and hyperpigmented macules.1 Some affected members also showed retarded growth and mental deficiency. The authors suggested that this complex of symptoms represented a new neurocutaneous syndrome distinct from tuberous sclerosis complex (TSC). We report a 9-year-old boy who was born with multiple hypopigmented and hyperpigmented macules distributed over the trunk and extremities. The skin lesions were well defined, asymmetrical, and not confined to any dermatome (Fig. 1a). Mucous membranes were spared. Otherwise the patient was healthy. His only brother, aged 3 years, had similar congenital skin lesions (Fig. 1b). This younger child was diagnosed in utero with symmetrical retarded growth, and during his first 3 years of life has shown growth retardation at percentile < 3

Renbök phenomenon in a patient with alopecia areata universalis

The Renbök phenomenon, or reverse Koebner phenomenon, was first reported in 1991 by Happle et al,1 who described 4 patients with extensive alopecia areata of the scalp with hair growth within plaques of psoriasis. Three additional cases have subsequently been reported,2-4 and the term was later extended to include patients with mosaic phenomena, one with alopecia areata without a nevus flammeus, and another without a congenital nevus.5,6 Whereas the Koebner phenomenon involves psoriasis of traumatic or inflammatory origin, the Renbök phenomenon occurs when some inflammatory process is inhibited by psoriasis

Neoadjuvant intralesional methotrexate in cutaneous squamous cell carcinoma: a comparative cohort study

Background: Intralesional methotrexate (MTX-il) has been used as neoadjuvant therapy for keratoacanthoma but has only been utilized in a few isolated cases of cutaneous squamous cell carcinoma as neoadjuvant therapy (cSCC). Objectives: The objective of this study was to evaluate the effectiveness in clinical practice of presurgical MTX-il infiltration to reduce the size of the cSCC. Safety and the impact on subsequent reconstructive surgical techniques was also assessment. Methods: Single, retrospective, observational study of two historical cohorts differentiated in time. Subjects included were diagnosed with infiltrating cSCC. Patients included in group-A received neoadjuvant MTX-il and patients included in group-B underwent scheduled surgery without prior infiltration. Univariate and multivariate analyses were performed. Results: Group-A patients (n = 43) showed an average reduction in the tumour area of 0.52 cm(2) , while in group-B (n = 43), the area increased by 0.49 cm(2) . A multivariate linear regression analysis demonstrated that MTX-il was the only independent variable that significantly reduced the tumour size [mean 42.6% (95% CI: 31.17-54.03)]. Tumours ≥2 cm in size required significantly a lower percentage of complex reconstructions (P = 0.026). Lower lip tumours showed a higher reduction in group treated with MTX-il (P = 0.045). The only complication observed was discomfort during methotrexate infiltration (60.47%). Conclusions: Neoadjuvant MTX-il reduced the presurgical size of cSCC lesions and could simplify their subsequent surgery

Factors influencing seasonal patterns of relapse in anti-TNF psoriatic responders after temporary drug discontinuation

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