Purpose of review To provide an update of vascular malformation syndromes by reviewing the most recent articles on the topic and following the new International Society for the Study of Vascular Anomalies (ISSVA) 2018 classification. Recent findings This review discusses the main features and diagnostic approaches of the vascular malformation syndromes, the new genetic findings and the new therapeutic strategies developed in recent months. Summary Some vascular malformations can be associated with other anomalies, such as tissue overgrowth. PIK3CA-related overgrowth spectrum (PROS) is a group of rare genetic disorders with asymmetric overgrowth caused by somatic mosaic mutations in PI3K-AKT-mTOR pathway that encompass a heterogeneous group of rare disorder that are associated with the appearance of overgrowth. CLOVES syndrome and Klippel–Trénaunay syndrome are PROS disease. Proteus syndrome is an overgrowth syndrome caused by a somatic activating mutation in AKT1. CLOVES, Klippel–Trénaunay and Proteus syndromes are associated with high risk of thrombosis and pulmonary embolism. Hereditary hemorrhagic telangiectasia is an autosomic dominant disorder characterized by the presence of arteriovenous malformations. New therapeutic strategies with bevacizumab and thalidomide have been employed with promising results.

IMPORTANCE Sexual health is a major aspect of life. Increasing scientific evidence shows a potential association between psoriasis and sexual dysfunction (SD) and erectile dysfunction (ED).OBJECTIVE To evaluate the available scientific evidence regarding epidemiologic associations and treatment outcomes between psoriasis and SD and ED.EVIDENCE REVIEW Information sources were MEDLINE and Embase databases, using the Scopus search engine. The search was performed on August 25, 2017. Search terms were psoriasis and sexual or sexual dysfunction, and the search was limited to epidemiologic studies published in English answering any of the 4 research questions. Quality was assessed according to the Centre for Evidence-Based Medicine.FINDINGS Twenty-eight studies representing 52 520 cases of psoriasis and 1 806 022 controls were included for review. Of the 28 studies, 19 were cross-sectional, 3 were clinical trials, 3 were quasi-experimental, 2 were population-based cohort, and 1 was population-based case-control. Prevalence of SD and ED ranged from 40.0% to 55.6% and 34.2% to 81.1%, respectively. Two of 2 studies observed an association between psoriasis and SD after adjusting for physical and psychological comorbidities. Five of 8 studies observed an independent association between ED and psoriasis. Among patients with psoriasis, the features that showed the strongest association with SD were anxiety and depression (5 of 5 studies), psoriatic arthritis (3 of 4 studies), and genital psoriasis (5 of 7 studies). Regarding ED, anxiety and depression (2 of 2 studies) and increasing age (3 of 3 studies) showed the strongest association. All 3 clinical trials using biologic drugs showed an improvement in SD compared with placebo.CONCLUSIONS AND RELEVANCE Patients with psoriasis have physical and psychological comorbidities that have been associated with a higher risk of SD. In addition, psoriasis may play a role in its development. The presence of anxiety, depression, psoriatic arthritis, genital lesions, and increasing age should raise the awareness of SD. Biologic drugs have demonstrated the improvement of SD in patients with psoriasis.

Background/Aims: TNF-α inhibitors represent the most advanced approved therapeutic option for moderate and severe forms of hidradenitis suppurativa (HS). However, in recent years, cases of paradoxical HS secondary to the use of these biological drugs have been described, with very few cases reported in the literature. The aims of this study are (1) to present 2 new cases of paradoxical HS and (2) to perform a systematic review of scientific evidence regarding paradoxical HS with TNF-α inhibitors. Material and Methods: This is a retrospective study in which we searched all the cases of paradoxical HS secondary to the use of TNF-α inhibitors published in the literature and included two additional cases observed in our clinical practice. Results: A total of 34 patients under TNF-α inhibitor treatment were included (adalimumab = 21; infliximab = 9; etanercept = 4). The median delay from exposure to TNF-α inhibitor and the development of paradoxical HS was 12 months (range 1–72). The majority of patients were Hurley stage II (58.8%). Clinical improvement and complete remission were more frequent when the TNF-α inhibitor was stopped or switched to another biological agent with a different therapeutic target rather than maintenance or change to another TNF-α inhibitor. Conclusions: Paradoxical HS is an unusual adverse effect of TNF-α inhibitors. When this adverse effect appears, interruption or substitution of treatment is associated with a better clinical outcome.

Corticosteroid infiltrations of lesions in hidradenitis suppurativa (HS) appear to be beneficial to acute flares. The aim of this study is to evaluate the effectiveness and safety of ultrasound-assisted intralesional corticosteroid infiltrations to HS lesions. Prospective cohort study between February 2017 and February 2019 on patients with mild to severe HS and one or more inflammatory lesions. The study intervention was ultrasound-assisted intralesional infiltration of triamcinolone acetonide 40 mg/ml. The main outcome was the complete response rate of infiltrated lesions versus non-infiltrated lesions. Two hundred and forty-seven infiltrated inflammatory lesions and 172 non-infiltrated lesions were included. At week 12, 81.1% (30/37) of nodules, 72.0% (108/150) of abscesses and 53.33% (32/60) of draining fistulas presented complete response versus 69.1% (47/68), 54.3% (38/70) and 35.3% (12/34) respectively for the non-infiltrated lesions. The Hurley stage negatively correlated with complete response for abscesses and draining fistulas at − 0.17 (SD 0.06) p < 0.01 and − 0.30 (SD 0.13) p < 0.02 respectively. Ultrasound-assisted corticosteroid infiltration is a useful technique for the treatment of inflammatory HS lesions, with high and sustained response rates, especially for abscesses and small to medium-size simple draining fistulas. The likelihood of response correlates negatively with the Hurley stage.

<b><i>Background:</i></b> New integrative hidradenitis suppurativa (HS) lesion pattern phenotypes have been proposed, an inflammatory phenotype (IP) and a follicular phenotype (FP). They are characterized by different lesion patterns, symptoms, and risks of disease progression. <b><i>Objectives:</i></b> To evaluate whether lesion pattern phenotypes (1) have a different cardiovascular risk factor profile, and (2) are associated with a different therapeutic approach in the setting of an HS clinic. <b><i>Methods:</i></b> A retrospective cohort study was conducted on 233 patients with HS. They were classified according to lesion pattern phenotype criteria. Data regarding cardiovascular risk factors and treatment decisions were gathered. <b><i>Results:</i></b> One hundred and seventeen HS patients (50.21%) were classified as FP and 112 (48.07%) as IP. IP was associated with more severe disease and greater impairment of quality of life. Regardless of disease severity, patients with IP may have a higher cardiovascular risk, assessed according to higher C-reactive protein (CRP) levels (12.75 vs. 5.89, <i>p</i> = 0.059). The lesion pattern phenotype also influenced treatment decisions regardless of disease severity. Patients with IP were more likely to be treated with systemic corticosteroids and adalimumab, showing that lesion pattern phenotypes are associated with different therapeutic approaches. <b><i>Conclusions:</i></b> IP is associated with higher CRP values, suggesting a greater cardiovascular risk in these patients and also a different therapeutic approach. This information could help guide dermatologists in the management of HS patients and help to determine future treatment recommendations.