Mortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study.

INTRODUCTION: Bullous pemphigoid is the most common autoimmune bullous dermatosis. In recent years several studies have tried to identify the main factors of the disease related with an increased risk of death. The aim of this multicenter Italian study was to assess the risk score of death considering epidemiologic, clinical, immunological, and therapeutic factors in a cohort of patients affected by bullous pemphigoid and try to identify the cumulative survival up to 120 months. METHODS: We retrospectively reviewed the medical records of patients with bullous pemphigoid who were diagnosed between 2005 and 2020 in the 12 Italian centers. Data collected included sex, age at the time of diagnosis, laboratory findings, severity of disease, time at death/censoring, treatment, and multimorbidity. RESULTS: A total of 572 patients were included in the study. The crude mortality rate was 20.6%, with an incidence mortality rate of 5.9 × 100 person/year. The mortality rate at 1, 3, 5, and 10 years was 3.2%, 18.2%, 27.4% and 51.9%, respectively. Multivariate model results showed that the risk of death was significantly higher in patients older than 78 years, in presence of multimorbidity, anti-BP180 autoantibodies >72 U/mL, or anti-BP230 > 3 U/mL at diagnosis. The variables jointly included provided an accuracy (Harrel's Index) of 77% for predicting mortality. CONCLUSION: This study represents the first nationwide Italian study to have retrospectively investigated the mortality rates and prognostic factors in patients with bullous pemphigoid. A novel finding emerged in our study is that a risk prediction rule based on simple risk factors (age, multimorbidity, steroid-sparing drugs, prednisone use, and disease severity) jointly considered with two biomarkers routinely measured in clinical practice (anti-BP230 and anti-BP180 autoantibodies) provided about 80% accuracy for predicting mortality in large series of patients with this disease.

Chloracne: a case series on cutaneous expression of CYP1A1 as diagnostic biomarker.

Chloracne, also known as metabolizing acquired dioxin-induced skin hamartomas (MADISH), is a rare disfiguring disease related to dioxin exposure. There is a paucity of literature on the clinical manifestations and pathogenesis of chloracne/MADISH. The aim of this study was to assess the clinical features of this very unusual acneiform eruption and to explore the pathogenesis of the disease. This was a retrospective, observational report study was conducted on five patients belonging to the same nuclear family (father, mother and three children) and a relative (father's brother) living in the same house. Histopathological, immunohistochemical, laboratory and toxicological analyses were performed for all patients. The results suggest that CYP1A1 in human skin is a diagnostic biomarker in chloracne, and was positive for all the patients in our sample. Tetrachlorodibenzo-p-dioxin is the most investigated dioxin responsible for chloracne; however, several other agonists, whether dioxin-like or not, can activate the aryl hydrocarbon receptor. To our knowledge, this Italian case series is the first study to suggest polychlorinated biphenyls as a possible cause of an overstimulation of aryl hydrocarbons causing the consequent acneiform eruption.

Body mass index stratification in hospitalized Italian adults with congenital heart disease in relation to complexity, diagnosis, sex and age.

BACKGROUND AND AIMS: Adults with congenital heart disease (ACHD) are at risk of overweight and obesity, two major health problems, though underweight can be a negative prognostic factor too. Awareness of the body mass index (BMI) in ACHD is very limited. The present study describes the use and prevalence of BMI in Italian symptomatic hospitalized ACHD patients in relation to complexity by Bethesda system classification, diagnosis, sex and age. METHODS AND RESULTS: We classified 1388 ACHD patients, aged 18-69 years, on the basis of their BMI, and compared them to the Italian reference population. In our total ACHD population we found a significantly higher prevalence of underweight compared to the Italian reference population (6.34% vs 3.20%). ACHD women were more underweight than men. Underweight decreased with age. Overweight was significantly less frequent in the total ACHD population (26.73% compared to 31.70%) in the Italian reference population. Men were more likely to be overweight than women. In statistical terms obesity was similar in the Italian reference population (10.50%) and our ACHD population (9.58%). Both overweight and obesity increased with age. Results were comparable using a diagnostic anatomical-functional classification and the Bethesda system classification. CONCLUSIONS: In our cohort of ACHD the prevalence of underweight was double that of the Italian reference population. The prevalence of overweight was lower, while obesity was similar. Since BMI does not account for differences in body fat distribution, a future aim will be to quantify the visceral component of the adipose tissue in ACHD patients and examine their body composition in order to reflect their risk of acquired cardiovascular disease better, and either to maintain or achieve an adequate visceral component.

Pigmented fungiform lingual papillae: dermoscopic and clinical features.

The tongue is covered by fungiform, filiform and circumvallate papillae. Fungiform papillae may be mainly pigmented in dark-skinned individuals. A single-centre study aimed to examine the clinical and dermoscopic features of pigmented fungiform papulae of the tongue (PFPT) in children, and a concise review of the literature has been performed. The clinical and anamnestic data of eight children affected by PFPT visited at the Pediatric Dermatology Unit of Bologna between 2010 and 2017, and a systemic review of all studies of PFPT published on PubMed up to 31 August 2017 has been collected and analysed. The results of our data were consistent with the literature review: dark brown to black coloured pinhead papules or bumps were observed in all cases of PFPT, and three types of clinical patterns have been detected. Moreover, the dermoscopic examination showed a cobblestone-like distribution and rose petal pattern. PFPT could be associated with hyperpigmentation of other sites such as the proximal nail folds and gums, and an intrafamiliar transmission is also possible. Clinical and dermoscopic features of PFPT may help clinicians to recognize this ethnic, acquired and benign condition.

Nail involvement as a negative prognostic factor in biological therapy for psoriasis: a retrospective study.

BACKGROUND: Psoriasis (Pso) has a strong impact on quality of life and a positive association has been reported between nail psoriasis (NP) and more severe disease, together with a longer duration of skin lesions. The treatment of NP represents a challenge and biological therapy can be recommended for severe disease. OBJECTIVE: The first end point of this retrospective study was to evaluate the time to achieve Psoriasis Area Severity Index (PASI) 75 in patients with and without NP treated with biological therapy. The second end point was to evaluate the efficacy of biological therapy to improve NP. METHODS: A total of 127 patients (88 men and 39 women) with moderate to severe Pso referring to our Service between 2007 and 2014 were included. Inclusion criteria were age ≥18 years and a 24 week treatment. The outcome variable was achievement of PASI 75 at 24 weeks with and without NP. All patients were treated with topical therapy and one of four different biological treatments: adalimumab (44.09%), etanercept (18.11%), infliximab (13.39%) and ustekinumab (24.41%). Physical examinations were performed every 4 weeks, and at each visit, the clinician assessed the PASI and Nail Psoriasis Severity Index (NAPSI). RESULTS: At multivariate Cox regression analysis, a smaller proportion of patients with NP achieved PASI 75 at 24 weeks than patients without NP when adjusted for the epidemiological, clinical features and biological treatment received. With all biological drugs, the NAPSI score began to improve already after 8 weeks (from 18.53 at week 0-2.83 at week 24). CONCLUSION: Patients with NP reach PASI 75 more slowly than patients without NP. Clinicians should therefore consider that treatment with a biological agent may require a longer period before reaching a satisfying therapeutical goal. Nevertheless, adalimumab, infliximab, ustekinumab and etanercept demonstrated their equal effectiveness in reducing the NAPSI score.

Life experiences and coping strategies in adults with congenital heart disease.

Many adults with congenital heart disease (ACHD) have to face considerable psychosocial difficulties. The aim of this study was to explore the life experiences of ACHD patients, from when they become aware of having a condition, till after the open heart surgery they underwent. The study was conducted with the use of unstructured, in-depth interviews, performed on 11 patients (age ranging: 20 - 56 y) after they recovered from open heart surgery and a focus group, which included 16 participants (age ranging: 22 - 46 y). Both the interviews and the focus group were recorded, transcribed and analyzed according to Grounded Theory procedures. Our findings show that the condition of diversity is the core of the emotional experiences connected to ACHD. Feeling different and being perceived as being different are clearly interlinked and coping strategies adopted resulted as being influenced by this perception. This study also clearly outlines the importance of having an adequate perception of one's condition and the link between maladaptive coping strategies and an incorrect perception of one's heart condition. Results are discussed in order to promote psychosocial interventions within and outside of the hospital setting in order to improve the patients' emotional wellbeing.

A novel device for the study of antimicrobial activity by vapor-contact of volatile substances on food products.

A novel device for the study of antimicrobial activity by vapour contact of volatile substances have been designed. This "big size" system, made up in inert acrylic material, is furnished with a fan and a hot plate with the aim to have a quick evaporation of volatile substances. It is able to contain fruits or other food products under controlled atmosphere and it can simulate real condition of storage or as well real condition of food pre-treatment by antimicrobial volatile substances. Such system is suitable to perform both in vitro (disk diffusion test) and in vivo (exposure and testing of food products) experiments. To shed light on the behaviour of this chamber the concentration in the head space of several substances have been monitored by GC-MS analysis during the time. Both single (mono-terpene compounds) and mixture of terpenoids have been studied. Different behaviours have been founds depending on the starting molecules studied. Limonene, myrcene and eucalyptol, in single standard experiment, show a similar shape of head space concentration curve versus the time: the concentration increases at the beginning, then reaches a maximum and decreases until it reaches a plateau. In contrast linalool shows a head space concentration curve constant during the time, whereas mixtures of terpenes like myrcene and linalool show a concentration curve of vapour phase in agreement with Raloult's Law. The experiments carried out with Essential Oils (EOs) shows that in our system only more volatile fraction of EOs compose the vapour phase.

[Art therapy for hospitalised congenital heart disease patients: a method of psychological intervention at the IRCCS Policlinico San Donato Milanese Hospital]. / L'arteterapia per i pazienti con cardiopatia congenita: metodologia di intervento psicologico presso il Reparto di cardiologia, cardiochirurgia pediatrica e cardiopatie conge- nite dell'adulto, IRCCS Policlinico San Donato.

The current work is the presentation of a new project at the IRCSS San Donato Milanese University hospital, in the sphere of Psychocardiology. Hospitalised children and adolescents often face psychosocial difficulties and the psychological condition of their parents frequently has an impact on their wellbeing. A strong need to take care, beyond the mere cure, is necessary in the hospital settings - that is a need to pay attention also to psychological aspects apart from the medical ones. Art therapy could be an answer for this need: the literature has outlined its efficacy in hospital, also due to the higher inclination of children and adolescents toward creativity. By providing and analysing the drawings of 10 young patients with congenital heart disease (CHD), this study outlines how the art therapy program gives these patients the opportunity to freely and directly express fears and anxieties about medical procedures and their disease. Moreover, through the creation of a tangible product, psychologists can better evaluate the psychological troubles of young patients and provide them and their parents with more focused and personalized support. This study also focuses on the perception of the utility that parents have of this new therapeutic intervention, offered at the Department of Paediatric Cardiac Surgery, confirming that art therapy is perceived as being effective and is definitely a good instrument in helping to "take care" of children and adolescents suffering from CHD.