Overgrowth syndromes are characterized by global or localized disproportionate growth associated with other anomalies, including vascular malformations and neurological and/or visceral disorders. CLOVES (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal naevi, Scoliosis/Skeletal and spinal anomalies) is an overgrowth syndrome caused by mosaic activating mutation in gene PIK3CA, which gives rise to abnormal PI3K-AKT-mTOR pathway activation. These mutations are responsible for the clinical manifestations of the syndrome, which include low- and high-flow vascular malformations, thoracic lipomatous hyperplasia, asymmetric growth, and visceral and neurological disorders. These common anomalies are illustrated with figures from two personal cases. Identification of the clinical and genetic characteristics of CLOVES syndrome is crucial for the differential diagnosis with other overgrowth syndromes, such as Proteus or Klippel-Trenaunay (K-T) syndromes, and for the therapeutic management of the different anomalies. In this context, a new entity comprising different syndromes with phenotypic mutations in PIK3CA has been proposed, designated PIK3CA-related overgrowth spectrum (PROS), with the aim of facilitating clinical management and establishing appropriate genetic study criteria.
In Spain, with full confinement measures and coinciding with the pandemic, pediatricians and dermatologists have received, through teledermatology/teleconsultation and social networks, a barrage of diverse images, which have subsequently allowed us to approach some of them by direct physical examination of early and late skin manifestations associated with SARS-Cov-2 infection. We designed a retrospective, cross-sectional study to evaluate the dermatological care of all those patients under the age of 16 who consulted, in person or telematically, for acral lesions (chilblain-like or erythema multiforme-like) in the context of the Coronavirus disease (COVID-19) pandemic, since 15 March 2020 to 24 April 2020, both included in the health area of the Hospital Universitario San Cecilio de Granada. Of all the patients collected, 18 (66%) were male and the overall mean age was 14.44 years. All lacked a personal history of interest and denied previous episodes of chilblains or Raynaud's phenomenon/disease. The clinic was limited to purpuric lesions located on acral regions distributed on hands and feet. Dermatologists and pediatricians should be aware of the lesions associated with COVID-19 infection and their possible complications. It remains to be identified if there are different dermatological patterns in the pediatric and adult population.
Psoriasis is a chronic and recurrent inflammatory skin disease that affects 2.3% of the population in Spain. 1 The first drug regarding the anti-IL23 family to join our therapeutic arsenal has been guselkumab. guselkumab is an immunoglobulin monoclonal antibody G1 lambda (IgG1λ) human anti-interleukin 23 (IL-23). IL-23, a regulatory cytokine, affects differentiation, expan
Paradoxical reactions during treatment with biological agents may be defined as an appearance or exacerbation of a pathological condition that usually responds to this class of drug. Typical examples of paradoxical adverse effect are, among others, palmoplantar pustular and psoriasiform reactions or HS, in patients during a treatment of rheumatoid arthitis or IBD mainly. A few reports have been described an exacerbation of psoriasis1, palmoplantar pustular, or pustular psoriasis eruption with secukinumab. Marasca et al. highlights the immunological comperformed to evaluate its efficacy and safety in moderate-to-severe HS, which will presumably be completed on January 2019. Hidradenitis suppurativa has also been reported to be paradoxically induced in patients treated with TNFα blockers, such infliximab or adalimumab (Delobeau et al., 2016). In a recently published series of 25 cases (Faivre et al., 2016), complete resolution of paradoxical hidradenitis suppurativa occurred after treatment discontinuation or switching to another biological agent, whereas reintroduction of the same biological agent resulted in relapse in all cases. Few reports have been published in order to communicate a good response to secukinumab in patients diagnosed of HS (with or without overlap psoriasis) (Thorlacius, Theut Riis, & Jemec, 2018). Nevertheless, secukinumab can also be the trigger of a paradoxical HS, as the case reported. Marasca (Marasca et al., 2019) highlights the immunological complexity that may surround autoinflammatory diseases showing the potential double pathophysiological face of secukinumab in HS, describing a case of secukinumab-induced HS and a case of HS provoked by adalimumab treatment and controlled with secukinumab therapy. The activation of interferon type I or IL-1β, and others local cytokine balance modifications, may explain the occurrence of paradoxical HS. This hypothesis is mainly focused on treatments based on TNF blockers. A possible explanation would be an increasing of the concentration of TNF-alpha, IL-26, IL-29, and IFN-γ produced by T-cells, accompanied by the increasing of IL-12 and IL-23 produced by dendritic cells. The possible magnification of ICAM-1 and TGF-B signaling, would trigger hyperproliferative keratinocytes, and as a result, HS lesions. Real world evidence and results from randomized clinical trials with secukinumab for HS, will shed light on the real and main role that anti-IL-17 drugs play in this complex disease. From clinical and immunological HS context, new therapeutic frontiers will be opened providing useful information to improve the knowledge of the immunological pathways that cause it. ORCID Francisco J. Navarro-Triviño https://orcid.org/0000-0002-5454-3671Ricardo Ruiz-Villaverde https://orcid.org/0000-0002-0381-6174
Background Psoriasis is a chronic systemic disease that requires long-term management.Despite data on follow-up studies going back 5 years, little is known about the condition's sustainability based on patient profiles. The aim of this study was to analyze drug survival and discontinuation rates for secukinumab treatment under real-world conditions. Patients and MethodsPatients with moderate-to-severe plaque psoriasis treated in the dermatology department of five Spanish medical centers between 2015 and 2019 were included in our retrospective study. Drug survival was assessed with Kaplan-Meier analysis plots and multivariate regression. Results In total, 171 treated patients were retrospectively recorded and analyzed for 152 weeks (37.40% had been diagnosed with psoriatic arthritis [PsA]). The discontinuation rate in the PsA group was 14.10% vs. 12.10% among those who had no PsA. The mean survival time of discontinuation was 63 weeks for PsA vs. 65 weeks for no PsA (P = 0.913). Secukinumab's estimated mean survival in PsA patients was 86% (estimated mean survival time 130 weeks) vs. 88% (estimated mean survival time of 133 weeks) in non-PsA patients (P = 0.676).Conclusion The mean survival time of patients in secukinumab treatment was comparable in all patient profiles and better than the data found in clinical trials and real-life studies. Figure 2Kaplan-Meier plot of drug survival for secukinumab over 152 weeks according to: (a) psoriasis duration in years (less than 10 years, between 10 and 20 years and more than 20 years from PsO was diagnosed), (b) PASI basal (≤10, >10 ≤20, and >20), (c) bionaïve patients and non-bio-naïve patients (those previously treated with one or more biologics), and (d) according to BMI (≥18.99 ≤24.99, >25.00, ≤29.99, and ≥30). Censored patients (active) were defined as those who continued treatment. Noncensored patients (event) were those who interrupted treatment because of any of the above-mentioned causes [Colour figure can be viewed at wileyonlinelibrary.com] Pharmacology and Therapeutics Drug survival, discontinuation rates, and safety profile of secukinumab in real-world patients Ruiz-Villaverde et al. 638
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