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Hidradenitis Suppurativa (HS) is a chronic, Skin Immune-Mediated Inflammatory Disease (sIMID), with a high impact on Quality of Life (QoL). Data on clinical management, comorbidities, psychosocial burden, and psychiatric disorders in paediatric patients with HS is scarce. The aim of our study was to compare clinical characteristics and Patient-Reported Outcome Measures (PROMs) for Health-Related Quality of Life (HRQoL) in adult and paediatric patients with HS. Data were collected on 601 patients with HS, 60 of whom (10.0%) were paediatric. The psychological and HRQoL impact was generally higher in adults than in children, but it was noteworthy also in paediatric patients. Early diagnosis and interventions based on education for the patients and their families and psychological support are the key to optimal disease management and adherence. Our study underlines the need for screening for HS-associated psychiatric disorders and for using age-specific tools to evaluate HRQoL. A multidisciplinary approach involving several specialists is needed, as well as liaison with primary care specialists to improve care, and management of comorbidities and psychological issues in patients with HS.

Abstract Background Inherited epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of skin fragility disorders characterized by blister formation following minor trauma. Four major types are distinguished based on the level of cleavage within the skin. Most EB forms present severely disabling cutaneous and systemic signs and symptoms. Management relies on daily time-consuming and distressing topical medications, and symptomatic treatment of systemic findings. Disease manifestations, symptoms, and daily care strongly affect patient and caregiver quality of life (QoL). To date, there are two validated EB-specific questionnaires, the “Quality of Life in Epidermolysis Bullosa” (QOLEB) and the “Epidermolysis Bullosa Burden of Disease” (EB-BoD) for the evaluation of patient and family disease burden, respectively. The aim of our study was to develop an Italian translation of the two questionnaires and to pilot-test them. Methods The guidelines for translation and cross-cultural adaptation of health-related QoL measures were followed. Initially, two separate translations were generated for each questionnaire, and subsequently reconciled by an expert committee. This was followed by a back-translation process. The original texts and all translations underwent revision by the expert committee, resulting in definitive versions. The final versions were then tested in a pilot study involving cognitive debriefing in a group of 17 families, representative of all EB major types. Results The translation and reconciliation process led to minor changes to obtain semantic/idiomatic/cultural equivalence of the Italian versions with the original ones and to reconcile the questions with the answer options. The cognitive debriefing process showed a good understanding and did not require text modifications. Conclusions The Italian versions of the QOLEB and EB-BoD provide valuable tools in everyday clinical practice of reference centers, and they allow the participation in multicenter international real-life observational studies as well as in controlled clinical trials. They enable the identification of disease-specific psychological and socioeconomic challenges for EB patients and their families, guiding targeted interventions to ensure appropriate and timely care.

ObjectiveThe prevalence of anxiety and depression in patients diagnosed with Alopecia Areata (AA) is very high and this significant burden of psychological symptoms threatens the Health-Related Quality of Life (HRQoL) of affected patients. Indeed, AA often does not produce significant physical symptoms, but it nonetheless disrupts many areas of mental health. Clinical assessment of disease severity may not reliably predict patient's HRQoL, nor may it predict the patient's perception of illness. For this reason, considerable effort has been made to apply and develop measures that consider patient's perception and assess the HRQoL of individuals affected by AA. The aim of this multicentric study was to provide the Italian version of the Skindex-16AA and to evaluate its psychometric properties in a clinical sample of consecutive patients with moderate-to-severe AA.MethodsThis is a longitudinal, multicenter, observational study. Patients returned for follow-up visits at 4-, 12-, and 24-weeks. The analyses of the current work aimed to confirm the factorial structure of the Skindex-16AA. In the case of non-fit, an alternative structure for the model was proposed, using an Exploratory Graph Analysis and the Bayesian approach.ResultsThe sample was composed of 106 patients with AA. Alopecia Universalis was the most frequently diagnosed type of alopecia at all time points. The analyses on the Skindex-16AA revealed that a two-factor structure with eight items fit the data best (Bayesian Posterior Predictive Checking using 95% Confidence Interval for the Difference Between the Observed and the Replicated Chi-Square values = −6.246/56.395, Posterior Predictive P-value = 0.06), and reported satisfactory psychometric properties (i.e., internal consistency and convergent validity).ConclusionThe Skindex-8AA demonstrated optimal psychometric properties (i.e., convergent and construct validity, and test-retest reliability) measured in a sample of patients with AA, that may suggest that it is an appropriate tool to measure the HRQoL in AA patients. However, further studies are needed in order to confirm and tested other psychometric features of this tool.

Bullous pemphigoid (BP) is the most common autoimmune bullous disease, whose main autoantigens are hemidesmosomal components BP180 and BP230. Although recent studies found no association between COVID-19 vaccines and BP, since mass vaccinations started, more than 90 vaccine-associated BP cases have been reported. To find an agreement among real-life clinical observations and recent epidemiologic data, we further investigated this topic. A total of 64 patients with BP onset in 2021 were demographically, clinically, and serologically characterized: 14 (21.9%) vaccine-associated patients (VA) developed BP within 5 weeks from the first/second vaccine dose. VA and vaccine-non-associated (VNA) patients had similar demographics and clinical and immunological characteristics. Noteworthy, the monthly distribution of BP onset during mass vaccinations paralleled vaccine administration to the elderly in the same catchment area. Additionally, in 2021, BP onsets in April–May and June–July significantly increased (p = 0.004) and declined (p = 0.027), respectively, compared to the three years before vaccination campaigns (2018–2020). Interestingly, VA and VNA patients showed statistically significant differences in the use of inhalers and diuretics. Our findings suggest that the COVID-19 vaccine may constitute an accelerating factor that, together with other triggering factors, could act in genetically predisposed individuals with possible sub-clinical autoreactivity against BP antigens, slightly accelerating BP onset.

ObjectiveAssuming that the difference exist in the manifestation of psychological suffering among genders, the purpose of this review is to summarize the current knowledge on gender differences in vitiligo quality of life and psychological assessment.MethodsWe searched in PubMed, Scopus, and Web of Science databases for original articles in English language. Results were screened according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA checklist).ResultsThe study yielded 107 results; 12 articles have been evaluated as eligible. Each eligible study has been screened and analyzed. The study’s qualitative evaluation revealed that 8 papers were classifiable as satisfactory, 4 were classifiable as unsatisfactory. The agreement between the coders was high (% agreement = 84.6%; Cohen’s kappa = 0.79). All considered researches (100%) were cross-sectional studies, based on self-report questionnaires. From our analysis, women with vitiligo had a higher risk to experience lower quality of life, and worse mental health in a wide range of psychopathology symptoms than men. A wide heterogeneity of tools is used to investigate the quality of life and psychological symptoms among these patients.ConclusionUnfortunately, there are few explanatory models proposed in the literature to rationalize these findings. It will be important to investigate in further researches the specific influence of known risk factors for psychopathology in this population to better explore these phenomena.

BackgroundRecent studies are stressing the idea that the level of Hidradenitis Suppurativa (HS) severity does not always correspond to the same illness load. In fact, it was found that there was no significant association between Skindex-17 and clinical severity of HS, and that some items of the Skindex-17 might be more related to HS severity than others.ObjectiveThe aim of the current study was to explore the associations between different levels of clinical severity of HS (mild, moderate, severe) and the Skindex-17 single-item responses in a large sample of adults patients.MethodsA cross-sectional study with a sample include 547 consecutive HS patients, approaching for the first time the specific dermatologic evaluation. Eligible criteria included age ≥ 18 years, HS diagnosis formulated contextually by the same expert clinician, and providing informed consent. All participants completed the Skindex-17 and were evaluated for the disease clinical severity with the International Hidradenitis Suppurativa Severity Score System. A Network Analysis was conducted, which represents a powerful methodological approach in clinical research. It allows to study specific patterns and the structure of complex system in order to better understand how the elements of the system interact with each-other. Three different Network Analyses were conducted for each group of HS clinical severity by including the items of the Skindex-17 as the nodes of each network.ResultsAmong 547 patients, 40% (219) were female and mean age was of 32.70 (±11.41). Different patterns among items of the Skindex-17 for the three clinical severity groups emerged, meaning that in each group different items are more crucial than others. The psychosocial subscale of the Skindex-17 is the most relevant when assessing the Quality of Life of individuals with different levels of HS severity, however, with the progression of the disease, individuals place attention also on two different aspects of the symptoms subscale of the Skindex-17, irritation and pain, which in turn perfectly reflect the severity of HS, from a clinical perspective.ConclusionThese results provide new insights on the association between levels of HS severity and related Quality of Life, measured with the Skindex-17.

Dermoscopy is widely used for the diagnosis of skin cancers and it increases the accuracy of basal cell carcinoma (BCC) detection. The dermoscopic criteria of BCC have recently been updated and they can be divided into three categories: vascular, pigment-related, and nonvascular/nonpigmentrelated. The objective of our multicentre retrospective study was to report the validity of a new dermoscopic pigment-related feature utilized to detect pigmented BCC (pBCC) (i.e., brown homogeneous blotches (BHB)). Cases of pBCC were collected from the databases of IDI-IRCCS of Rome and from three Italian private dermatology centres. BHB were defined as the presence of circumscribed areas of brown homogeneous pigmentation which are devoid of any dermoscopic specific arrangement (net, fat fingers, etc.) and of other internal dermoscopic structures, except for occasional vascular ones, such as arborizing vessels or globules/dots. Controls included melanocytic and non-melanocytic lesions. We examined images of 270 pigmented lesions (female 145; 51.8%) including 90 histopathological proven pBCC and 180 control cases (i.e., 90 melanocytic and 90 non-melanocytic lesions). BHB were present in 61 cases of 90 pBCC. These results showed a sensibility of 67.8 and a specificity of 93.3 with a positive predictive value of 83.6, negative predictive value of 85.3, posLR 10.2, negLR 0.3; OR 29.4, P value

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Abstract Indoor tanning is associated with an increased risk of skin cancer. Nonetheless, its use is still widespread. We aimed to investigate the socio-demographic and clinical characteristics of sunbed users in a group of participants in the skin cancer prevention campaign organized by the Italian Cancer League (LILT). During almost 2 years, 4409 individuals were screened in 18 centers. Participants reported having used sunbeds before the age of 15 years in 2.2% of cases, while after age 15 the prevalence of use was 22.2%. Participants with complete information were 3692. Sunbed users aged > 15 years were significantly more frequently females, young, living in Northern Italy, highly educated, and current or former smokers. They had darker phototype, more common nevi, had used sunbeds more frequently before the age of 15, reported a history of sunburns, and use of sunscreens. Indoor tanning is an important public health issue and a relevant target for primary prevention. However, not all countries have adopted the recommendations issued by the World Health Organization (WHO) on health risks associated with artificial tanning. A deeper insight into the topic may contribute to identify the best prevention strategies.

Abstract Background Autosomal recessive congenital ichthyoses (ARCI) are a genetically heterogeneous group of rare and chronic disorders characterized by generalized skin scaling and hyperkeratosis, erythroderma, and palmoplantar keratoderma. Additional features include ectropion, eclabium, ear deformities, foul-smell, joints contractures and walking problems, recurrent infections, as well as pruritus and pain. No curative therapy is available and disease care mainly relies on daily application of topical emollients and keratolytics to the whole-body surface. Altogether, disease signs and symptoms and treatment modalities have a major impact on quality of life of patients and their caregivers. However, very few studies have evaluated the family disease burden in ARCI. Methods We have performed an Italian multicenter cross-sectional study to assess the secondary disease impact on family members of pediatric and adult patients with ARCI, using a validated dermatology-specific questionnaire, the family dermatology life quality index (FDLQI). Disease severity was assessed by the dermatologist in each center. Results Seventy-eight out of 82 patients who were accompanied by at least one family member filled the FDLQI. Forty-eight (61.5%) patients were aged less than 18 years. The mean FDLQI score was 10.3 (median 10), and the most affected dimensions were (1) time needed for care, (2) extra-housework, and (3) household expenditure. Higher total FDLQI score significantly correlated with more severe disease score (P = 0.003). Features associated with greater family burden included recurrent infections (P = 0.004), foul-smell (P = 0.009), palmoplantar keratoderma (P = 0.041), but also presence of scales on the face (P = 0.039) and ear deformities (P = 0.016). Conclusions Our findings highlight the major socio-economic and psychological burden imposed by ARCI on the QoL of family caregivers. In addition, they show that global evaluation of disease impact also on family members is an essential part of patient-reported outcomes. Finally, our data underline the need to develop specific measures for family support.

Abstract The coronavirus disease (COVID‐19), during its course, may involve several organs, including the skin with a petechial skin rash, urticaria and erythematous rash, or varicella‐like eruption, representing an additional effect of the severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection, as commonly observed in other viral diseases. Considering that symptomatic patients with COVID‐19 generally undergo multidrug treatments, the occurrence of a possible adverse drug reaction presenting with cutaneous manifestations should be contemplated. Pleomorphic skin eruptions occurred in a 59‐year‐old Caucasian woman, affected by a stable form of chronic lymphocytic leukemia, and symptomatic SARS‐CoV‐2 infection, treated with a combination of hydroxychloroquine sulfate, darunavir, ritonavir, sarilumb, omeprazole, ceftriaxone, high‐flow oxygen therapy devices, filgrastim (Zarzio®) as a single injection, and enoxaparin. The patient stopped all treatment but oxygen and enoxaparin were continued and the patient received a high‐dose Desametasone with complete remission of dermatological impairment in 10 days. It is very important to differentially diagnose COVID‐19 disease‐related cutaneous manifestations, where is justified to continue the multidrug antiviral treatment, from those caused by an adverse drug reaction, where it would be necessary to identify the possible culprit drug and to start appropriate antiallergic treatment.

Abstract Non-melanoma skin cancers include basal and squamous cell carcinoma. These tumors have become an important health issue for their high incidence and for the morbidity, especially if untreated for a long period. Over the last 20 years, therapeutic approaches for these tumours have been improved and tailored. In this survey we provided data from one hundred and ten Italian dermatologists regarding knowledge and attitude towards different therapeutic approaches on non-melanoma skin cancers. In our study, we observed that surgery and imiquimod 5% cream were the most used treatment by dermatologists for basal cell carcinoma, while, surgery was the most common treatment for cutaneous squamous cell carcinoma. Furthermore, we observed some differences regarding the prescribed therapies in the different Italian geographical areas (i.e., Mohs’ surgery and electrochemotherapy were more frequently used in Northern compared to Central and Southern Italy whereas immunotherapy was more used in Southern compared to Northern and Central Italy) and even considering the year of specialization of the dermatologists (i.e., immunotherapy with cemiplimab was prescribed mainly by dermatologists with 10–19 years of specialization). However, for locally advanced and metastatic forms of basal and squamous cell carcinoma, Hedgehog Pathway Inhibitors and anti- Programmed cell death protein antibody treatment, respectively, were used in line with the newest evolution of therapies regarding this topic. Considering the importance of skin cancers and its progressive increase in incidence, it is crucial to improve the knowledge of different therapeutic approaches among dermatologists.

ObjectivesNon-melanoma skin cancers (NMSC) include two main types: basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (cSCC). Generic Health-Related Quality of Life (HRQoL) instruments revealed little to no HRQoL impairment in NMSC patients. Instead, the use of specific skin disease HRQoL tools contradicted those observations. For example, the Skin Cancer Index (SCI) was suggested as a validated instrument for the evaluation of the impact of skin cancers on HRQoL, and has already been validated in several languages, but not in Italian. The aim of this study is to testing some psychometric properties of the Italian version of the SCI questionnaire in a large sample of NMSC patients.MethodsThis is a cross-sectional, single-center, observational study. Firstly, different factor models proposed in the literature were compared and the model with the best fit was identified. Secondly, the psychometric properties of the SCI, convergent validity and reliability, were evaluated.ResultsThe sample was composed of 371 NMSC patients. The factor analysis revealed that a revised version of the original model had the best fit [χ2(df = 85) = 354.53, p

ObjectivesTo measure general psychopathology in dermatologic outpatients using the Symptom-Checklist-K-9 (SCL-K-9); to investigate whether the SCL-K-9 is able to categorize patients with and without significant non-psychotic disorders; and to perform a single-item analysis of the SCL-K-9, with a focus on gender differences.MethodsCross-sectional study on consecutive dermatological patients. We used two self-administered questionnaires to assess general psychopathology symptoms: General Health Questionnaire-12 (GHQ-12) and SCL-K-9. Sociodemographic information was collected with standardized forms. The performance of the SCL-K-9 in classifying patients according to their current emotional distress severity was assessed using a ROC procedure. Finally, we measured differences in scores obtained among women and men in SCL-K-9 single items.ResultsA total of 292 patients were studied (71.2% women). We observed statistically significant differences in SCL-K-9 total mean scores and in most single items among genders. We found that it would be more appropriate to use gender-specific cut-offs when using SCL-K-9 to screen dermatological patients for general psychopathology.ConclusionThe SCL-K-9, with its compact format could provide, in a short time, a wide range of information related to critical areas that challenge the mental health of patients with skin diseases.

Sarcoidosis is a multisystem disease that affects the skin in 20 to 30% of cases. Skin findings are often the initial presenting signs, and cutaneous sarcoidosis may appear with a wide variety of lesions; it is often considered an imitator of many other skin diseases. Clinical appearance and specific dermoscopic criteria, confirmed by a typical pathology, may guide to the correct diagnosis. We report a case of a man affected by maculo-papular sarcoidosis on the back, in which the detection of cutaneous lesions was the initial step to determine the systemic nature of the disease.

Introduction: Shellfish allergy is an important cause of food allergies worldwide. Both in vivo and in vitro diagnostics failure nowadays is caused by the poor quality of the extracts associated with the scarce availability of allergenic molecules in the market. It is known that not all patients with shellfish allergies experience adverse reactions to mollusks. It is still unclear how to detect and diagnose these patients correctly. Aim: To investigate the features of shrimp-allergic patients either reactive or tolerant to mollusks, with the currently available diagnostic methods. Methods: Nineteen centers, scattered throughout Italy, participated in the real-life study, enrolling patients allergic to shrimp with or without associated reactions to mollusks. Patients underwent skin tests using commercial extracts or fresh raw and cooked shrimp and mollusks, and IgE reactivity to currently available allergenic extracts and molecules was measured in vitro. Results: Two hundred and forty-seven individuals with a self reported adverse reactions to shrimp participated in the study; of these 47.8% reported an adverse reaction to mollusks ingestion (cephalopod and/or bivalve). Neither of the tests used, in vivo nor in vitro, was able to detect all selected patients. Accordingly, a great heterogeneity of results was observed: in vivo and in vitro tests agreed in 52% and 62% of cases. Skin tests were able to identify the mollusk reactors (p

Non-melanoma skin cancers (NMSCs), which include basal cell carcinoma (BCC), squamous cell carcinoma (SCC), and actinic keratosis (AK), are the most common cancer diseases in the Caucasian race. If diagnosed late and improperly treated, BCC and SCC can become locally advanced and metastasize. Malignant melanoma (MM) is less frequent but more lethal than NMSC. Given the individual and social burdens of skin cancers, performing an adequate prevention is needed. Ultraviolet (UV) ray exposure is one of the main risk factors for skin cancer. Thus, the first-choice prevention strategy is represented by photoprotection that can be both topical and systemic. The latter consists of the oral administration of molecules which protect human skin against the damaging effects of UV rays, acting through antioxidant, anti-inflammatory, or immunomodulator mechanisms. Although several compounds are commonly used for photoprotection, only a few molecules have demonstrated their effectiveness in clinical trials and have been included in international guidelines for NMSC prevention (i.e., nicotinamide and retinoids). Moreover, none of them have been demonstrated as able to prevent MM. Clinical and preclinical data regarding the most common compounds used for systemic photoprotection are reported in this review, with a focus on the main mechanisms involved in their photoprotective properties.

Background: Human subcutaneous dirofilariasis caused by Dirofilaria repens is a vector-borne zoonotic disease mostly transmitted from dogs to humans through a mosquito’s blood meal. Heartwarms replication is amplified by the climate change, the increase of the range of suitable vectors, the facilitation of pet travel and the high rate of undiagnosed dirofilariasis in dogs. Observation: We describe a case of a young Romanian woman, resident in Rome for 18 years, that came to our attention for the appearance for five months of a subcutaneous nodule in the left arm. The patient reported that she first felt an insect bite, after which she noticed the onset of an erythematous and itchy wheal in the same skin area, turned into a subcutaneous nodule within a few weeks. The ultrasound examination showed a hypoechoic subcutaneous formation of 1,2 cm in diameter, containing a ribbon-like structure made up of hyperechoic parallel double lines, reminiscent of a warm. Based on this suspicion, we opted for the surgical radicalization of the lesion. The histological examination confirmed the radiological hypothesis of a warm-like foreign body morphologically compatible with Dirofilaria repens. Key message: Our experience shows how a clinical nonspecific skin nodular lesion may conceal an unexpected and unsettling diagnosis of subcutaneous Dirofilaria repens.

Actinic keratosis (AK) is considered a pre-cancerous lesion that may progress into an invasive squamous cell carcinoma. Its prevalence is increasing, and it has been estimated worldwide to range between 1% and 44% of the adult population. The main risk factors for AK are advanced age, fair skin phototype and cumulative sun exposure. Treatment of AK consists of lesion-directed treatment to target single lesions (i.e., cryotherapy, curettage, electrocoagulation, and laser therapy) or field therapy to treat multiple AKs (i.e., photodynamic therapy (PDT), 5- Fluorouracil (5-FU), diclofenac sodium (DIC), imiquimod (IMQ), ingenol mebutate (Ing Meb)). The choice of therapy depends on the number and localization of AKs, patient’s condition, and the patient's tolerability and compliance. In this survey we provided data from one hundred and ten Italian dermatologists regarding knowledge and attitude towards different therapeutic approaches specific for AK. In our study, we observed that the most frequent treatments for AK are cryotherapy and PDT while surgery and laser therapy are the less frequently utilized. The most frequent topical therapies utilized are DIC and IMQ 3.75% cream compared to IMQ 5% cream, Ing Meb, and 5-FU. Choice the correct treatment for AK can be challenging but the adherence to therapy plays a key role to obtain good results. Considering the high and progressive increase in incidence of AK, it is crucial to improve the knowledge of different therapeutic approaches among dermatologists.

Objective: Alexithymia is frequent in patients with some chronic dermatological conditions. The aim of the study was to measure the prevalence of alexithymia in patients with systemic sclerosis (SSc) using two different tools.Methods: Consecutive patients diagnosed with SSc were recruited at day hospital and hospitalization regimen. Alexithymia was measured using the self-administered questionnaire 20-item Toronto Alexithymia scale (TAS-20) and the semi-structured interview 24-item Toronto Structured Interview for Alexithymia (TSIA).Results: The study sample consisted of 67 female patients, aged from 29 to 82 years. According to TAS-20, 22.4% of patients were alexithymic and 17.9% were borderline alexithymic. Also, in our sample mean TAS-20 value was 48.9 and TSIA mean value was 20.3. Spearman’s correlation coefficient between TAS-20 total score and TSIA total score was 0.603. A high correlation was observed between TAS total score and the “Affective Awareness” (AA) scale of the TSIA. TSIA total score significantly correlated with the “Difficulty describing feeling to others” (DDF) scale of the TAS-20. The highest correlation between scales of the two instruments was that between TAS-20 DDF and TSIA AA (r = 0.675).Conclusion: The prevalence of alexithymia in SSc patients was higher than in the general population, and similar to that of psoriasis patients. The measurements obtained using the TAS-20 and the TSIA were highly correlated. However, they also showed some diversities in the scales, indicating that they may measure different domains.

Hidradenitis Suppurativa (HS) is a chronic skin disease involving intimate and sensitive areas and affecting physical and mental health. We investigated the prevalence of sexual desire and functioning impairment, and their associations with quality of life, anxiety, depression, minor psychiatric disorders (MPD), and clinical features (e.g., disease severity) in 77 patients with HS who completed self-report measures and answered to questions assessing socio-demographic characteristics, lifestyle habits, and hindered sexuality due to HS. The majority of patients reported hindered sexuality, and poor sexual functioning, while showing good levels of dyadic and solitary sexual desire. No associations were found between clinical severity and sexuality measures. Multivariate analyses showed significant associations of sexual outcome measures with alcohol consumption, low Body Mass Index, family history of HS, and severe skin symptoms. Moreover, we found that the presence of negative psychological factors (i.e., MPD, anxiety, poor mental status) increased the risk of sexual impairment. These findings underline the important role of psychological and sexual aspects in HS patients and suggest that physicians should consider the effect of disease burden on patients' sexual health.

The term non-melanoma skin cancer (NMSC) refers to skin cancer different from melanoma, and it is usually restricted to basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and their pre-cancerous lesions, e.g., actinic keratosis. These conditions represent the most frequent tumors in Caucasians and are characterized by an increasing incidence worldwide and a high socio-economic impact. The term Integrated Care Pathway (ICP) refers to “a complex intervention for the mutual decision making and organization of care processes for a well-defined group of patients during a well-defined period”. The purpose of this paper is to present a proposal from the Italian Association of Hospital Dermatologists (ADOI) for an ICP organization of care of NMSC, considering the hub-and-spoke model in the different geographical areas. This proposal is based on the most recent literature and on documents from the Italian Association of Medical Oncology (AIOM), the European consensus-based interdisciplinary guidelines from the European Association of Dermato- Oncology (EADO), and the National Comprehensive Cancer Network (NCCN). We initially discuss the NMSC outpatient clinic, the role of the multidisciplinary working groups, and the hub-and-spoke model regarding this topic. Then, we define the ICP processes specific for BCC and SCC. The ICP for NMSC is an innovative strategy to guarantee the highest possible quality of health care while the hub-andspoke model is crucial for the organization of different health care structures. Considering the importance on this topic, it is essential to create a valid ICP together with an efficient organization within the different geographical areas.

Autosomal recessive congenital ichthyoses (ARCI) are characterized by generalized skin scaling, hyperkeratosis, erythroderma, and disabling features affecting the skin (palmoplantar keratoderma, fissures, pain, itch), eyes, ears, and joints. Disease severity and chronicity, patient disfigurement, and time and costs required for care impose a major burden on quality of life. This multicentre cross-sectional study investigated the impact of ARCI on quality of life of patients and families, using the Dermatology Life Quality Index (DLQI), the Children DLQI (CDLQI) and Family Burden of Ichthyosis (FBI) questionnaires. Disease severity was assessed by a dermatologist. A total of 94 patients were recruited, of whom 52 (55.3%) children. Mean age was 20.1 (median 13.5) years. The mean CDLQI/DLQI score was 7.8, and 21 patients scored >10, indicating a major impairment in quality of life: symptoms, feelings and treatment problems were the most affected domains of quality of life. FBI showed a major repercussion on psychological factors and work. The results of this study highlight the impact of ARCI on specific aspects of patient and family life, underlining the need for psychological support.

Abstract Background Inherited ichthyoses are rare disorders characterized by generalized skin scaling. Among them, autosomal recessive congenital ichthyoses (ARCI) form a major subgroup presenting lifelong and severely disabling cutaneous and extracutaneous features and symptoms for which no curative treatment is available. Management relies on daily time-consuming and distressing topical medications. Disease manifestations, symptoms, and daily care affect not only the patient self-perception, but also different dimensions of patient and family life. To date, there is only a French validated ichthyosis-specific questionnaire, “Family Burden in Ichthyosis” (FBI), for the evaluation of family disease burden. It addresses economical aspects, daily life, familial and personal relationships, work, and psychological impact. The aim of our study was to develop an Italian translation of the French FBI questionnaire and to pilot-test it in ARCI patients. Methods The guidelines for cross-cultural adaptation of health-related quality of life measures were followed. Specifically, two independent forward translations were produced, followed by a reconciliation step by a multidisciplinary expert committee and back-translation. Revision of the original text and all translations was performed by the expert committee leading to a final version, which was pilot-tested by cognitive debriefing on 10 caregivers whose comments were evaluated by the committee. Results The translation and reconciliation process led to minor changes in five items in order to clarify the questions in relation to the possible answers or to obtain semantic/idiomatic/cultural equivalence of the Italian version with the French one. The cognitive debriefing process resulted into further minor wording modifications in four items to describe more precisely the disease impact according to parents’ comments. The FBI developer approved the final Italian FBI version. Conclusions The Italian version of the FBI generated in the present study is a useful instrument to measure the impact of ichthyosis on family daily life, education and working activities, psychological implications, and the disease economic load. The questionnaire will be further validated through a multicenter Italian study on burden of ARCI. A validated Italian questionnaire is a valuable tool for future clinical trials. In addition, it can be used to rapidly identify family distressing situations, which require attention and prompt intervention.

The difference between men and women in the impact of keratinocyte carcinomas on quality of life has not been widely studied. This study of 364 patients with keratinocyte carcinoma, measured quality of life using the self-administered 12-item Short Form Health Survey (SF-12) and Skindex-29. Results for both the physical and the mental components of SF-12 were worse in women than in men. For the mental component, women had significantly lower scores compared with men in almost all subgroups, based on demographic and clinical variables. The Skindex-29 emotions mean score was worse in women than in men. Women reported significantly higher level of worry that the disease could get worse and of developing scars, and more depression. On the other hand, men reported lower quality of sleep. The impact of keratinocyte carcinomas on quality of life is generally higher in women than in men. Such data may be important for tailored management of the disease in different categories of patients.

Depression is frequent in patients with hidradenitis suppurativa. However, its relationship with quality of life and clinical severity needs further investigation. In this cross-sectional study, 341 adult, consecutive patients with hidradenitis suppurativa completed the 12-item General Health Questionnaire (GHQ-12), which has been shown to be able to identify cases of major depressive disorder in dermatological patients. The frequency of depression in hidradenitis suppurativa patients was 29.0%. In patients with depression, severity (International Hidradenitis Suppurativa Severity Score System (IHS4)), quality of life (Skindex-17; Dermatology Life Quality Index (DLQI)), and health status (36-item Short Form Health Survey (SF-36)) were significantly worse compared with patients with no depression. The highest linear correlation was observed between GHQ-12 and the psychosocial scale of the Skindex-17 and the SF-36 mental scale. In contrast, correlation between GHQ-12 and clinical severity was poor. Depression is an important comorbidity in hidradenitis suppurativa, which is strongly associated with impairment in quality of life, but not linearly correlated with clinical severity.

In this study we examined whether aspects of positive functioning [reappraisal, sense of coherence (SOC), and positivity] were associated with spiritual well-being and psychological distress after controlling for negative functioning (skin-related symptoms, type of disease, and expressive suppression) in individuals with skin diseases. We also examined whether negative functioning aspects were linked to spiritual well-being and distress when controlling for aspects of positive functioning. The study used a cross-sectional design with a sample of 192 individuals with psoriasis and systemic sclerosis (SSc) (Mage = 51.6 years, SD = 16.5). Two hierarchical multiple regressions were conducted to determine whether spiritual well-being and psychological distress were accounted for by skin-related symptoms, type of disease, emotion regulation strategies, SOC, and positivity. Positivity was the most important contributor to better spiritual well-being, followed by both the comprehensibility/manageability and meaningfulness SOC subscales, after controlling for the other variables. High skin-related symptoms and expressive suppression were associated with lower psychological distress, whereas high SOC and reappraisal correlated with higher psychological distress. The findings of this study pave the way for further research on how SOC and positivity may reduce the effects of both skin-related symptoms and emotion dysregulation and facilitate spiritual well-being of individuals with skin diseases. Interventions aimed to enhance inner resources of these individuals and help them to find a meaning in their experience of skin disease might reduce psychological distress and improve spiritual well-being. Our findings suggest that healthcare professionals should consider positive functioning aspects in future interventions for individuals with skin diseases.

Pain and discomfort are important symptoms in dermatology. The aim of this cross-sectional, multicentre study was to describe the prevalence of pain/discomfort and its associations in patients with several dermatological conditions across 13 European countries. The outcome was the prevalence of pain/discomfort according to a question of the EQ-5D questionnaire. Data collected from November 2011 to February 2013 were complete for 3,509 consecutive outpatients. Moderate or extreme pain/discomfort was reported by 55.5% of patients and 31.5% of controls with no skin conditions. The highest proportions were reported by patients with hidradenitis suppurativa (92.9%), leg ulcer (81.4%), prurigo (80%) and lichen planus (75.6%). Pain/discomfort was associated with older age, low educational level, clinical severity, flare on scalp or hands, itch, depression, anxiety, low quality of life, and thoughts of suicide. It is important to enquire specifically about pain/discomfort during clinical consultations and to address it when planning a patient’s care.

Merkel cell carcinoma (MCC) is a rare and extremely aggressive neuroendocrine carcinoma of the skin, with increasing incidence worldwide. This review intends to propose a comprehensive evaluation of MCC epidemiology, clinical features, pathogenetic mechanisms, diagnosis, and therapies. A section is dedicated to immunological aspects and another to the involvement of angiogenesis and angiogenic growth factors in MCC progression, proposing novel diagnostic and therapeutic approaches. Advanced MCC tumors have been treated with immune checkpoint inhibitors with effective results. Therefore, the state of art of this immunotherapy is also examined, reporting on the most recent clinical trials in the field. We conclude by underlining the achievements in the understanding of MCC pathology and indicating the present needs for effective diagnosis and therapeutic management of the disease.

Introduction: The Integrated Care Pathway (ICP) represents a multidisciplinary outline of anticipated care, placed in an appropriate timeframe, to support patients with specific conditions or symptoms. The aim of this paper is to define the ICP for patients with melanoma referring to the “Istituto Dermopatico dell’Immacolata-IRCCS di Roma e Villa Paola” (“Center”). Methods and results: A multidisciplinary group (oncologists, dermatologists, surgeons, pathologists etc.) was defined as well as a facilitator to act as a link between all experts. The first step of ICP development was a review of current practice for patients with melanoma referring to the Center. This first step had the scope to define the multidisciplinary process map (a “picture” of the care plan) for patients with melanoma. The process map defined: i) the activities performed during delivery of care to the patients, ii) the responsibilities for these activities and iii) potential problem areas or opportunities for improvements. The process map formed the basis of the final ICP document. Conclusion: The adoption of melanoma ICP will allow the multidisciplinary group to ensure that clinical guidelines and available evidence are incorporated into everyday practice. (Oncology, HTA & Market Access)

Cutaneous squamous cell carcinoma (cSCC), a non-melanoma skin cancer, is a keratinocyte carcinoma representing one of the most common cancers with an increasing incidence. cSCC could be in situ (e.g., Bowen’s disease) or an invasive form. A significant cSCC risk factor is advanced age, together with cumulative sun exposure, fair skin, prolonged immunosuppression, and previous skin cancer diagnoses. Although most cSCCs can be treated by surgery, a fraction of them recur and metastasize, leading to death. cSCC could arise de novo or be the result of a progression of the actinic keratosis, an in situ carcinoma. The multistage process of cSCC development and progression is characterized by mutations in the genes involved in epidermal homeostasis and by several alterations, such as epigenetic modifications, viral infections, or microenvironmental changes. Thus, cSCC development is a gradual process with several histological- and pathological-defined stages. Dermoscopy and reflectance confocal microscopy enhanced the diagnostic accuracy of cSCC. Surgical excision is the first-line treatment for invasive cSCC. Moreover, radiotherapy may be considered as a primary treatment in patients not candidates for surgery. Extensive studies of cSCC pathogenic mechanisms identified several pharmaceutical targets and allowed the development of new systemic therapies, including immunotherapy with immune checkpoint inhibitors, such as Cemiplimab, and epidermal growth factor receptor inhibitors for metastatic and locally advanced cSCC. Furthermore, the implementation of prevention measures has been useful in patient management.

Background: Systemic photoprotection (i.e., administration of substances such as nicotinamide, carotenoids, and vitamin D) may be important to reduce photocarcinogenesis or to support long-term protection against UV irradiation. Clinical trials showed that oral nicotinamide is effective in reducing the onset of new nonmelanoma skin cancers (NMSCs), while other oral photoprotectors failed to achieve the reduction of new melanoma or NMSC formation in humans. The aim of this study was to summarize the current state of knowledge of systemic photoprotection and to evaluate the knowledge and attitude of dermatologists regarding these treatments. Methods: The survey was conducted on a sample of dermatologists recruited according to a snowball sampling procedure. The questionnaire consisted of a first part asking for characteristics of the participant and a second part with 12 specific questions on their knowledge about systemic photoprotection, particularly their knowledge of astaxanthin, β-carotene, nicotinamide, and vitamin D3. Results: One hundred eight dermatologists answered the survey. Most of them (85.2%) stated that oral photoprotectors have a role in the prevention of skin cancer, and responses mainly mentioned nicotinamide. More than half of them (54.6%) had prescribed all the considered oral photoprotectors, but the majority of them had prescribed nicotinamide, mainly for 2 to 3 months during summer, almost invariably (n = 106) associated with topical photoprotectors. Most dermatologists (>80%) were aware of scientific publications demonstrating an effect of systemic photoprotectors on NMSC. Conclusions: Most Italian dermatologists have positive views on oral photoprotection in skin cancer and are aware of the demonstrated potential of nicotinamide in the prevention of NMSCs.

Basal cell carcinoma (BCC) is the most common human cancer worldwide, and is a subtype of nonmelanoma skin cancer, characterized by a constantly increasing incidence due to an aging population and widespread sun exposure. Although the mortality from BCC is negligible, this tumor can be associated with significant morbidity and cost. This review presents a literature overview of BCC from pathophysiology to novel therapeutic approaches. Several histopathological BCC subtypes with different prognostic values have been described. Dermoscopy and, more recently, reflectance confocal microscopy have largely improved BCC diagnosis. Although surgery is the first-line treatment for localized BCC, other nonsurgical local treatment options are available. BCC pathogenesis depends on the interaction between environmental and genetic characteristics of the patient. Specifically, an aberrant activation of Hedgehog signaling pathway is implicated in its pathogenesis. Notably, Hedgehog signaling inhibitors, such as vismodegib and sonidegib, are successfully used as targeted treatment for advanced or metastatic BCC. Furthermore, the implementation of prevention measures has demonstrated to be useful in the patient management.

The original article contains a misspelling of co-author, Christine Bodemer’s name which is presented correctly in this Correction article.

Background Sézary syndrome is a rare and very aggressive leukemic variant of cutaneous T-cell lymphoma characterized by extensive skin involvement and a malignant circulating CD4+ T-cell clone which homes to the skin, over-expresses CD60, and lacks CD7, CD26 and CD49d. So far prognostic markers in this disease are limited to treatment with systemic steroids, age, serum lactate dehydrogenase, and a white blood cell count of 20×109/L or higher: no other biological marker with prognostic value, especially related to malignant cells, has been described.Design and Methods We used flow activated cell sorting analysis to compare the distribution of the T-cell receptor-Vβ repertoire and several surface molecules (CD7, CD26, CD49d and CD60) within the circulating CD4+ T-cell population in 62 patients with Sézary syndrome, 180 with mycosis fungoides, 6 with B-cell lymphomas, and 19 with chronic eczema. We calculated the 5-year overall survival of patients with Sézary syndrome after first hospital admission using Kaplan–Meier product–limit estimates and hazard ratios from the Cox proportional hazards model.Results We found that both higher number of CD60+ and lower number of CD49d+ cells within circulating CD4+ T cells at disease presentation were significantly associated with a lower probability of survival. An exceedingly high risk of death was observed for patients with a combination of a high proportion of CD4+CD60+ cells (≥ 0.5×109/L) and low proportion of CD4+CD49d+ cells (