Search

Atopic dermatitis (AD) is one of the most common diseases worldwide. Severe AD has a major impact on the quality of life of patients. We performed a systematic literature review on the epidemiology of AD in Italian pre-school children (age 0–5 years) and we assessed the available data on the severity of AD. In August 2022, we performed a bibliographic search using PubMed/Medline and EMBASE. We identified 10 studies with Italian data on the prevalence and/or incidence of AD in pre-school children. The period (12 months) prevalence of AD varied widely across studies, ranging between 4.0% and 42.2%, with median estimates of 14.3% among all studies and 11.8% among studies from 2010 onwards. Applied to the Italian population, this leads to a prevalence of 309,000–375,000 pre-school AD cases. Only one study computed the incidence of AD, reporting rates of 9 cases per 100 person-years in children aged 0–1 year, and 2.5 cases per 100 person-years in children aged 1–4 years. Severity data from Italy were also reviewed, across three identified three studies. A point estimate found 8.4% of cases were considered severe in one study based on the Patient-Oriented Eczema Measure (POEM), with an overall range of 7.8–11% across different Italian studies and according to various severity score types. [ABSTRACT FROM AUTHOR]

Introduction: Biological therapies are valuable treatments for severe psoriasis. Children aged under 12 years are underrepresented in therapeutic trials for these drugs. The objective of the 'BiPe Jr' cohort study was to evaluate the drug survival, effectiveness, tolerance and switching patterns of biological therapies in children under 12 years of age with psoriasis. Methods: We conducted a multicentre retrospective study of children with psoriasis who received at least one injection of a biological agent, even off-licence, before the age of 12 years in France and Italy, collecting the data between April and August 2021. The data collected were from March 2012 up to August 2021. Results: In total, 82 children (mean age: 9.1 years; females: 61.0%) received 106 treatments. The drugs administered were adalimumab (n = 49), etanercept (n = 37), ustekinumab (n = 15), anakinra (n = 2), infliximab (n = 2) and secukinumab (n = 1). The most common form of psoriasis was plaque psoriasis (62.9%). The Physician Global Assessment and the Psoriasis Area Severity Index (PASI) scores decreased significantly from baseline to 3 months after treatment initiation for the three main biological drugs; PASI went from 14.1 ± 9.4 to 4.1 ± 11.3 for adalimumab (p = 0.001), 14.9 ± 9.3 to 5.1 ± 4.0 for etanercept (p = 0.002) and 11.6 ± 8.3 to 2.6 ± 2.2 for ustekinumab (p = 0.007). A trend towards higher 2-year maintenance rates was observed for ustekinumab and adalimumab, compared with etanercept (p = 0.06). 52 children discontinued their biological therapy, most frequently due to inefficacy (n = 28) and remission (n = 14). Seven serious adverse events (SAEs) were reported, including four severe infections. Discussion: Our analyses of drug survival and treatment patterns, combined with those of previous studies conducted in older children, indicate that there is a trend towards higher 2-year survival rates of ustekinumab and adalimumab. The SAEs identified were rare, but highlight the need for increased vigilance concerning infections. Overall, the biological therapies showed good effectiveness and safety profiles when used in daily practice for the treatment of young children with psoriasis. [ABSTRACT FROM AUTHOR]

Melorheostosis (MEL) is an uncommon, sclerosing disease, characterised by hyperostosis of long bones, resembling the flowing of candle wax. The disease is sporadic and the pathogenesis is still poorly understood. Occasionally, the same family can include individuals with MEL and Osteopoikilosis (OPK), a disease characterised by multiple round foci of increased bone density. LEMD3 gene mutations are related to OPK and Buschke-Ollendorff Syndrome, a genetic condition in which an association between MEL, OPK and skin lesions is observed. In rare cases, LEMD3 mutations and recently mosaic MAP2K1 gene mutations have been correlated to MEL suggesting that somatic mosaicism could be causative of the disease. In this study, we described the clinical, radiological and molecular findings of 19 individuals with MEL and 8 with OPK and compared the results to the medical literature. The molecular analyses of this case series corroborate the available data in the medical literature, indicating that LEMD3 germline mutations are not a major cause of isolated MEL and reporting five further cases of OPK caused by LEMD3 germline mutations. [ABSTRACT FROM AUTHOR]

Aim: We collected 'real-life' data on the management of patients with mastocytosis in the Italian Mastocytosis Registry.Methods: Six hundred patients diagnosed with mastocytosis between 1974 and 2014 were included from 19 centers.Results: Among adults (n = 401); 156 (38.9%) patients were diagnosed with systemic mastocytosis. In 212 adults, no bone marrow studies were performed resulting in a provisional diagnosis of mastocytosis of the skin. This diagnosis was most frequently established in nonhematologic centers. In total, 182/184 pediatric patients had cutaneous mastocytosis. We confirmed that in the most patients with systemic mastocytosis, serum tryptase levels were >20 ng/ml and KIT D816V was detectable.Conclusion: The Italian Mastocytosis Registry revealed some center-specific approaches for diagnosis and therapy. Epidemiological evidence on this condition is provided. [ABSTRACT FROM AUTHOR]

This article provides comprehensive recommendations for the systemic treatment of severe pediatric psoriasis based on evidence obtained from a systematic review of the literature and the consensus opinion of expert dermatologists and pediatricians. For each systemic treatment, the grade of recommendation (A, B, C) based on the treatment's approval by the European Medicines Agency for childhood psoriasis and the experts' opinions is discussed. The grade of recommendation for narrow-band-ultraviolet B phototherapy, cyclosporine, and retinoids is C, while that for methotrexate is C/B. The use of adalimumab, etanercept, and ustekinumab has a grade A recommendation. No conventional systemic treatments are approved for pediatric psoriasis. Adalimumab is approved by the European Medicines Agency as a first-line treatment for severe chronic plaque psoriasis in children (≥ 4 years old) and adolescents. Etanercept and ustekinumab are approved as second-line therapy in children ≥ 6 and ≥ 12 years, respectively.Conclusion: A treatment algorithm as well as practical tools (i.e., tabular summaries of differential diagnoses, treatment mechanism of actions, dosing regimens, control parameters) are provided to assist in therapeutic reasoning and decision-making for individual patients. These treatment recommendations are endorsed by major Italian Pediatric and Dermatology Societies. What is Known: • Guidelines for the treatment of severe pediatric psoriasis are lacking and most traditional systemic treatments are not approved for use in young patients. Although there has been decades of experience with some of the traditional agents such as phototherapy, acitretin, and cyclosporine in children, there are no RCTs on their pediatric use while RCTs investigating new biologic agents have been performed. What is New: • In this manuscript, an Italian multidisciplinary team of experts focused on treatment recommendations for severe forms of psoriasis in children based on an up-to-date review of the literature and experts' opinions. [ABSTRACT FROM AUTHOR]

Background: Despite not being licensed for the treatment of infantile hemangiomas (IH) in infants younger than 5 weeks or older than 5 months, propranolol is often used in these age groups to prevent or to treat potentially severe complications. The objective of the present study was to review the experience of 8 Italian pediatric and dermatologic centers regarding propranolol treatment for IH started before 5 weeks or after 5 months of age. Methods: We retrospectively reviewed the records of patients followed up for IH, on propranolol treatment started before 5 weeks or after 5 months of age, and collected information on sociodemographic data, treatment indications, IH involution, IH relapse, and treatment side effects. Results: A total of 343 patients were enrolled; 15 were started on propranolol before 5 weeks (group 1), 328 were started after 5 months of age (group 2). The most frequent indications were permanent aesthetical disfigurement (91. 8%) and function threatening complications (42.6%). In most cases, the treatment was effective. The involution was partial in 67.7% of patients. In 11.8% of cases a relapse was observed. No relapse was observed in group 1. Treatment complications were reported in 15.8% of children, most frequently sleep disorders (6.6%), followed by irritability (5.1%) and diarrhea (2.2%). Only a case of mild constipation was observed in group 1. Conclusion: The safety and effectiveness profile of propranolol in infants younger than 5 weeks or older than 5 months may be acceptable. Taking in account propranolol's potential in preventing severe complications, further studies should assess the acceptability of propranolol treatment, especially in the <5-week age group. [ABSTRACT FROM AUTHOR]

Background: Families of children affected with atopic dermatitis (AD) often report fear and anxiety regarding treatment with topical corticosteroids (TCS), which may lead to reduced compliance. The objective of our study was to measure, through a standardized questionnaire, fear of TCS in families of pediatric patients with AD and to identify items associated with fear. Methods: Families of pediatric patients with AD were enrolled in 9 Italian centers of pediatric dermatology. Enrolled parents were invited to fill in a questionnaire including questions on sociodemographic and clinical characteristics and 3 sets of questions on corticosteroid phobia (general fear, specific fears, behaviours regarding TCS). Determinants of the level of general fear were investigated through multivariable analysis. Results: A total of 300 outpatients with AD were enrolled. Most parents (80%) had a high instruction level. Eighty-one percent reported to have a certain amount of fear of TCS. At the multivariable analysis, fear of TCS was associated with the following items: believing that TCS treatment advantages do not overweight disadvantages (P = 0.011); believing that TCS may be dangerous independently from the specific side effect (P < 0.001). Moreover, TCS fear was associated with fear of applying too much cream (P = 0.001). Conclusion: TCS phobia is widespread among Italian families of children with AD. Fear of TCS is associated with fear of applying too much cream, thus increasing the risk of poor compliance and treatment failure. Therapeutic education of families on the use of TCS should be implemented. [ABSTRACT FROM AUTHOR]

A group of Italian experts in impetigo medical care sought to define 10 statements to describe the ideal characteristics of the best local antibiotic treatments, and to provide relevant information re- garding their appropriate use and prescription that should be considered in clinical practice for impetigo management. A group of Italian experts in impetigo medical care sought to define 10 statements to describe the ideal characteristics of the best local antibiotic treatments, and to provide relevant information regarding their appropriate use and prescription that should be considered in clinical practice for impetigo management. A consensus on ideal features of antibiotic therapy for the treatment of impetigo was appraised by an online Delphi-based method, based on a panel of 61 infectious disease specialists, pediatricians, and dermatologists coordinated by a scientific committee of 5 experts specializing in impetigo management. Full or very high consensus was reached on the 10 statements identified to describe the characteristics of the best hypothetic antibiotic therapy for impetigo together with indications for appropriate antibiotics use. Several criteria have to be considered when selecting topical antibacterial therapy for impetigo. Beyond efficacy and safety, antimicrobial susceptibility and pharmacological characteristics of the agent are essential points. Formulation of the antimicrobial product is fundamental, as well as patient and caregiver preference, to facilitate therapeutic adherence, to achieve the infection control, and to obtain the best benefit from treatment (Curr Ther Res Clin Exp. 2023; 84:XXXXXX). [ABSTRACT FROM AUTHOR]

Background Atopic dermatitis (AD) is a chronic inflammatory skin disease and can be the first step of the atopic march. Objective In this retrospective study, we analysed the immunological and clinical patterns of AD in a group of children affected by the disease since their first years of life, in order to evaluate if and how these patterns can change over time, and to identify biomarkers that can possibly correlate with the clinical phenotype. Methods We enrolled Caucasian children with diagnosis of AD performed by a specialist on the basis of Hanifin and Rajka's criteria and with a first clinical and laboratory evaluation before 5 years of age. Patients were divided in 2 groups: IgE-associated AD (with or without allergic respiratory diseases) and non-IgE-associated AD. Results Among 184 patients enrolled in this study, at the beginning 30/184 were classified as having non-IgE-associated AD, but during follow-up, at the median age of 36 months, 15 patients became allergic. All 15 patients who switched from the non-IgE to the IgE-associated form had a significantly earlier onset of AD than those who did not switch. Dust mite sensitization seem to be the best biomarker (OR 2.86) to predict the appearance of allergic respiratory diseases. Conclusion IgE-associated AD is more frequent in childhood than non-IgE-associated AD. These two phenotypes are different in the age of onset and in the remission patterns. In the first years of life, it is important to distinguish the different phenotypes in order to evaluate possible allergic related conditions. [ABSTRACT FROM AUTHOR]

We performed a retrospective study covering a 10-year period (January 1997-January 2007) on children referred to our unit or followed up for Becker's nevus (BN). The aim of this study was to better define the characteristics of BN in childhood, when this hypermelanosis is more frequent than the limited number of studies might suggest. Our data show that BN in children has predilection sites similar to those in adults, but hypertrichosis is rarer. Sun exposure does not appear to play an etiopathogenetic role. The higher incidence in men reported in adult case studies was not found in children. During and after puberty, androgenic stimulation is known to play a pathogenetic role in BN, especially in men, but our cases were mostly younger than 6 years old. [ABSTRACT FROM AUTHOR]

To analyze the clinical features, response to treatment, and follow-up of lichen striatus and any associated symptoms or disease, we designed a retrospective study involving 115 affected children at the Pediatric Dermatology Unit of the Department of Dermatology of the University of Bologna, Bologna, Italy. Between January 1989 and January 2000 we diagnosed lichen striatus in 37 boys and 78 girls (mean age 4 years 5 months). We studied their family history and the season of onset, morphology, distribution, extent, duration, histopathology, and treatment of their lichen striatus. We found that family history was negative in all our patients except for two pairs of siblings. The majority of children had the disease in the cold seasons; precipitating factors were found in only five cases. The most frequently involved sites were the limbs, with no substantial difference between upper and lower limb involvement. When lichen striatus was located on the trunk and face, it always followed Blaschko lines; in seven children the bands on the limbs appeared to be along the axial lines of Sherrington. In 70 cases, lichen striatus was associated with atopy. The mean duration of the disease was 6 months and relapses were observed in five children, and in one instance the disease had a prolonged course. Only a few case study series of lichen striatus in children have been reported and ours is the largest to date. The etiology of lichen striatus remains unknown in the majority of our patients. The confirmed association with atopy observed in our patients may be a predisposing factor. It has generally been accepted that lichen striatus follows the lines of Blaschko, and this distribution is a sign of both a topographic and a pathogenetic concept. In patients where lichen striatus is along axial lines, a locus minoris resistentiae, we suppose that this distribution may only be an illusory phenomenon in instances in which the trigger factor prefers this route, consisting of several successive Blaschko lines, but appearing as a single band. [ABSTRACT FROM AUTHOR]

Background: Since the COVID-19 pandemic started, great interest has been given to this disease, especially to its possible clinical presentations. Besides classical respiratory symptoms, dermatological manifestations occur quite often among infected and non-infected patients, particularly in children. A prominent IFN-I response, that is generally higher in children compared to adults, may not only cause chilblain lesions, but it could also prevent infection and viral replication, thus justifying the negative swab results, as well as the absence of relevant systemic symptoms in positive cases. Indeed, reports have emerged describing chilblain-like acral lesions in children and adolescents with either proven or suspected infection., Methods: Patients aged from 1 to 18 years old were enrolled in this study from 23 Italian dermatological units and were observed for an overall period of 6 months. Clinical pictures were collected along with data on the location and duration of skin lesions, their association with concomitant local and systemic symptoms, presence of nail and/or mucosal involvement, as well as histological, laboratory and imaging findings., Results: One hundred thirty-seven patients were included, of whom 56.9% were females. Mean age was 11.97±3.66 years. The most commonly affected sites were the feet (77 patients, 56.2%). Lesions (48.5%) featured cyanosis, chilblains, blisters, ecchymosis, bullae, erythema, edema, and papules. Concomitant skin manifestations included maculo-papular rashes (30%), unspecified rashes (25%), vesicular rashes (20%), erythema multiforme (10%), urticaria (10%) and erythema with desquamation (5%). Forty-one patients (29.9%) reported pruritus as the main symptom associated with chilblains, and 56 out of 137 patients also reported systemic symptoms such as respiratory symptoms (33.9%), fever (28%), intestinal (27%), headache (5.5%), asthenia (3.5%), and joint pain (2%). Associated comorbid conditions were observed in 9 patients presenting with skin lesions. Nasopharyngeal swabs turned out positive in 11 patients (8%), whereas the remainder were either negative (101, 73%) or unspecified (25, 18%)., Conclusions: COVID-19 has been credited as the etiology of the recent increase in acro-ischemic lesions. The present study provides a description of pediatric cutaneous manifestations deemed to be potentially associated with COVID-19, revealing a possible association between acral cyanosis and nasopharyngeal swab positivity in children and teenagers. The identification and characterization of newly recognized patterns of skin involvement may aid physicians in diagnosing cases of asymptomatic or pauci-symptomatic COVID patients.

Background: There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience., Methods: A multicenter retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units., Results: We collected the data of 69 children affected by: circumscribed morphea (39.1%); linear morphea of trunk and limbs (14.5%); en coupe de sabre morphea (ECDS) (14.5%); progressive facial hemiatrophy (8.7%); generalized form (18.8%); mixed morphea (4.4%). The mean age at onset was 6.86±3.21 years, mainly between 2 and 8 years, but is statistically significantly lower for ECDS (4.5±3.03). Localizations were: head/neck (30.4%), limbs (26.1%), trunk (14.5%), 2 or more sites (29%), most often the trunk plus limbs. Extracutaneous manifestations were observed in 26.1% patients. 10 patients presented a second autoimmune disorder. Treatments were topical in 26.1% cases and systemic (alone or associated with topical treatments) in 68.1%., Conclusions: There was a lack of uniformity in the management of patients and an increasing awareness of dermatologists on the use of systemic therapies, in particular of methotrexate, which is no longer exclusive to rheumatologists. Methotrexate causes stabilization and improvement of the clinical signs, but topical creams are still considered adjuvant or maintenance therapies during/after the use of systemic drugs.

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.

Atopic dermatitis (AD) is a chronic inflammatory disease with increasing global incidence, which has a multifactorial pathogenesis and a variable expressivity. Clinical features of AD are different in adults compared to children, but it is well recognized the substantial impact of the disease on patients' quality of life at any age. Indeed, little is known about AD in adolescence, a period of life generally associated with high psychological burden and vulnerability to depression. Guidelines for the management of AD are available for both children and adults but specific guidelines for the diagnosis and treatment of AD in adolescents are lacking. Seven Italian scientific societies of dermatologists, allergists, and pediatric allergists joined in a specific meeting to provide practical guidance for the diagnosis and management of moderate-to-severe adolescent AD suitable for the Italian clinical practice. Through a modified Delphi procedure, consensus was reached by 59 Italian experts in the management of AD on 20 statements covering five areas of interest about adolescent AD, including disease complexity, burden and social impact, diagnosis and definition of severity, current treatments, and new biologic therapies. This paper reports recommendations for the diagnosis and management of AD specifically in adolescents, pointing out some peculiar clinical features and focusing on the choice of medications. Dupilumab, the first biologic approved for the treatment of adolescents with AD, represents a useful treatment option due to its efficacy and reassuring safety profile.

Alopecia areata (AA) is an organ-specific autoimmune disorder that targets anagen phase hair follicles. The course is unpredictable and current available treatments have variable efficacy. Nowadays, there is relatively little evidence on treatment of AA from well-designed clinical trials. Moreover, none of the treatments or devices commonly used to treat AA are specifically approved by the Food and Drug Administration. The Italian Study Group for Cutaneous Annexial Disease of the Italian Society of dermatology proposes these Italian guidelines for diagnosis and treatment of Alopecia Areata deeming useful for the daily management of the disease. This article summarizes evidence-based treatment associated with expert-based recommendations.

The aim of this guidance is to provide recommendations to clinicians and other interested parties on chronic urticaria in children. The Italian Society for Pediatrics (SIP), the Italian Society for Allergy and Immunology (SIAIP), the Italian Society for Pediatric dermatology (SIDerP) convened a multidisciplinary panel that prepared clinical guidelines for diagnosis and management of chronic urticaria in childhood. Key questions on epidemiology, natural history, diagnosis, and management were developed. The literature was systematically searched and evaluated, recommendations were rated and algorithms for diagnosis and treatment were developed. The recommendations focus on identification of diseases and comorbidities, strategies to recognize triggering factors, improvement of treatment by individualized care.