Hidradenitis suppurativa in patients with Down syndrome.

Literature data about hidradenitis suppurativa (HS) in patients with Down syndrome (DS) are limited. In this retrospective study, 6 (4M/2F; mean age: 18.7 years) out of 313 patients affected by HS (1.9%) had DS. The age of appearance of HS was from 9 to 14 years. No family history of HS was present. BMI ranged from 20.1 to 29.9. In all patients, HS was located below the diaphragm. Five out of six patients were staged as Hurley II severity.

HIDRAdisk: validation of an innovative visual tool to assess the burden of hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, relapsing, inflammatory skin disease characterized by painful inflamed nodules, recurrent abscesses and fistulas located in apocrine gland-bearing body sites. The negative impact of HS on patient's quality of life (QoL) has been reported to be greater than other dermatologic conditions as psoriasis and atopic eczema, and its improvement is an important goal in disease management. Nowadays, there are no specific validated QoL instruments available for HS and generic dermatologic questionnaires are used. OBJECTIVE: The objective of this study was to demonstrate the validity, reliability and responsiveness of HIDRAdisk, a new innovative tool designed for rapid assessment of HS burden and, at the same time, an intuitive graphic visualization of the measurement outcome. METHODS: A multicentre, longitudinal, observational study was conducted to validate the HIDRAdisk compared with other validated questionnaires [Skindex-16, Dermatology Life Quality Index (DLQI), Work Productivity and Activity Impairment-General Health (WPAI:GH)] and to evaluate its correlation with disease severity in Italian patients with any degree of HS severity, as measured by Hurley stage and HS Physician Global Assessment (HS-PGA). RESULTS: A total of 140 patients (59% women; mean age 34.9 ± 11.0 years) were enrolled in 27 dermatologic centres. HIDRAdisk showed a strong correlation with Skindex-16 and DLQI, and a good one with WPAI:GH (correlation coefficient: 0.7568, 0.6651 and 0.5947, respectively) and a statistically significant correlation with both Hurley stage and HS-PGA. Very good internal consistency (Cronbach coefficient >0.80; intraclass correlation coefficient >0.6), with correlation between the 10 items, good test-retest reliability (Spearman correlation coefficient, 0.8331; P < 0.0001) and responsiveness to changes were demonstrated. CONCLUSION: Our study shows that HIDRAdisk, a short and innovative visual HS QoL instrument, has been psychometrically validated in Italian language and it may help improve the management of HS once implemented in routine clinical practice.

Autoimmune bullous diseases in non-HIV Kaposi's sarcoma: a retrospective study in a large cohort of patients.

BACKGROUND: Kaposi's sarcoma (KS) is a rare endothelial neoplasm caused by the human herpesvirus 8 (HHV-8). Its risk is increased in immunocompromised patients, including those undergoing immunosuppressive therapy for autoimmune bullous diseases. Conversely, HHV-8 infection has been hypothesized to be a triggering factor of bullous diseases, especially pemphigus. Given the fact that both KS and autoimmune bullous diseases have a low incidence in the general population, it could be expected that the association between these disorders would be exceptional. OBJECTIVES: To assess the frequency of bullous diseases in a large cohort of non-HIV KS patients and to describe our experience concerning the clinical features, natural history and treatment options in this setting. METHODS: We performed a retrospective review of all patients with non-HIV KS in association with bullous disease followed at our department between 1990 and 2016. Medical records were reviewed for demographics, medical history, clinical characteristics and treatment. RESULTS: Among 1362 patients with classic or iatrogenic KS, 14 (1.03%) also suffered from bullous disease. The mean age at diagnosis of both disorders was 85.8 years with a male/female ratio of 9 : 5. Among these 14 cases, nine (0.66%) were associated with bullous pemphigoid (BP), three (0.22%) with localized BP and two (0.15%) with pemphigus vulgaris. Seven had developed a bullous disease after being diagnosed with KS, while in the remaining seven cases, KS developed after the onset of bullous disease. As expected, KS worsened when corticosteroids were used. CONCLUSION: Bullous diseases seem to be more frequent among patients with KS, supporting the hypothesis that HHV-8 may be involved in their pathogenesis. Therapeutic management of these cases should take into account KS-inducing potential of corticosteroids.

Treatment of mild to moderate acne with a fixed combination of hydroxypinacolone retinoate, retinol glycospheres and papain glycospheres.

AIM: A fixed combination of 0.1% hydroxypinacolone retinoate (synthetic esther of 9-cis-retinoic acid), 1% retinol in glycospheres and 2% papain in glycospheres in aqueous gel has been recently introduced into the Italian market in order to reduce the incidence and severity of irritant contact dermatitis caused by topical retinoids, without compromising their efficacy. Primary objectives of this sponsor-free, pilot, open, multicenter study were to evaluate the efficacy and tolerability of this gel in patients with comedonal-papular, mild to moderate acne of the face. METHODS: Ninety-eight Caucasian patients (28 males and 70 females), with an age ranging from 15 to 40 years, were treated with the gel once daily for 12 weeks. Acne severity and treatment efficacy were evaluated by means of the Global Acne Grading System (GAGS) and lesions count. RESULTS: Ninety-four patients were considered evaluable. A 41% mean reduction in the GAGS score was observed; a 40.8% mean reduction of total lesions was recorded; 15.3% of patients experienced mild to moderate local side effects (dryness, peeling, erythema, burning). No patients stopped the treatment because of these side effects. CONCLUSION: This study, based on a high number of evaluable patients, demonstrates that this fixed combination is an effective and safe option for the treatment of comedonal-papular, mild to moderate acne of the face. A controlled clinical study is necessary to confirm these data.

Upper limb muscular activity and perceived workload during laryngoscopy: comparison of Glidescope(R) and Macintosh laryngoscopy in manikin: an observational study.

BACKGROUND: The interaction between operators and their working environment during laryngoscopy is poorly understood. Numerous studies have focused on the forces applied to the patient's airway during laryngoscopy, but only a few authors have addressed operator muscle activity and workload. We tested whether different devices (Glidescope(®) and Macintosh) use different muscles and how these differences affect the perceived workload. METHODS: Ten staff anaesthetists performed three intubations with each device on a manikin. Surface electromyography was recorded for eight single muscles of the left upper limb. The NASA Task Load Index (TLX) was administered after each experimental session to evaluate perceived workload. RESULTS: A consistent reduction in muscular activation occurred with Glidescope(®) compared with Macintosh for all muscles tested (mean effect size d=3.28), and significant differences for the upper trapezius (P=0.002), anterior deltoid (P=0.001), posterior deltoid (P=0.000), and brachioradialis (P=0.001) were observed. The overall NASA-TLX workload score was significantly lower for Glidescope(®) than for Macintosh (P=0.006), and the factors of physical demand (P=0.008) and effort (P=0.006) decreased significantly. CONCLUSIONS: Greater muscular activity and workload were observed with the Macintosh laryngoscope. Augmented vision and related postural adjustments related to using the Glidescope(®) may reduce activation of the operator's muscles and task workload.

Rehabilitation Modulates High-Order Interactions Among Large-Scale Brain Networks in Subacute Stroke.

The recovery of motor functions after stroke is fostered by the functional integration of large-scale brain networks, including the motor network (MN) and high-order cognitive controls networks, such as the default mode (DMN) and executive control (ECN) networks. In this paper, electroencephalography signals are used to investigate interactions among these three resting state networks (RSNs) in subacute stroke patients after motor rehabilitation. A novel metric, the O-information rate (OIR), is used to quantify the balance between redundancy and synergy in the complex high-order interactions among RSNs, as well as its causal decomposition to identify the direction of information flow. The paper also employs conditional spectral Granger causality to assess pairwise directed functional connectivity between RSNs. After rehabilitation, a synergy increase among these RSNs is found, especially driven by MN. From the pairwise description, a reduced directed functional connectivity towards MN is enhanced after treatment. Besides, inter-network connectivity changes are associated with motor recovery, for which the mediation role of ECN seems to play a relevant role, both from pairwise and high-order interactions perspective.

Comparison between directed causal flow metrics for the assessment of resting-state EEG motor network connectivity in subacute stroke patients.

Isolated effective coherence (iCoh) is a measure of neural causal functional connectivity from EEG signals that was proven to overperform the Generalized Partial Directed Coherence (gPDC). However, iCoh sensitivity in the identification of reliable functional neural connections with respect to random links was not investigated. This study aims to compare the sensitivity of iCoh and gPDC with a statistical surrogates' approach. The cerebral motor network topology of a cohort of subjects in sub-acute stage after stroke was investigated. iCoh showed enhanced statistical discriminative power of the relevant connections within the motor network with respect to gPDC. This property influenced the assessment of ipsilesional intra-hemispheric topographic variations occurring in the population after a physical rehabilitation program.