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Background and objective: IgA vasculitis (IgAV), a predominantly pediatric leukocytoclastic disease, has an unpredictable, though largely benign, evolution. The aim of this study was to retrospectively investigate any potential clinical or laboratory predictors of gastrointestinal involvement in a single-center cohort of children with IgAV. Patients and methods: A total of 195 children with a history of IgAV, regularly followed-up for an average period of 1 ± 2.6 years via outpatients clinics of the pediatric rheumatology unit in our University, were assessed, analyzing their clinical and laboratory variables in relationship with their disease evolution and outcome. Results: Univariate analysis showed that a higher neutrophil granulocyte count and lower lymphocyte count (expressed as a percentage of the total white blood cells) were significantly associated with the presence of gastrointestinal involvement at the first examination (65.2 ± 13% versus 58.8 ± 12%, p = 0.02, and 26.4 ± 11% versus 32.1 ± 11%, p = 0.02, respectively). A positive pharyngeal swab for Streptococcus pyogenes, a deficiency of 25-hydroxyvitamin D, a persistence of purpuric rash for more than 1 month, and purpuric lesions in the genital area were also associated with gastrointestinal involvement (p = 0.0001, p = 0.0001, p = 0.007 and p = 0.001, respectively). However, multiple logistic regressions with correction for the patients’ sex and age showed that lower 25-hydroxyvitamin D levels, persistent rash, and genital lesions were independently and significantly associated with signs of gastrointestinal involvement. We then performed a secondary analysis (both univariate and multivariate) to investigate whether vitamin D deficiency was associated with other IgAV manifestations: we found that only 25-hydroxyvitamin D deficiency remained significantly associated with gastrointestinal involvement in IgAV. Conclusions: Patients with IgAV and vitamin D deficiency might be more prone to developing gastr

BACKGROUND: In the last decades diagnosis of dermatological diseases has achieved a significant progress with the aid of imaging technologies. In pediatric population dermatologic procedural investigations require special considerations, skill sets, and knowledge. Avoiding unnecessary invasive procedures in children is highly recommended to reduce psychological disturbance and cosmetical scars. Line-field confocal optical coherence tomography (LC-OCT) is an innovative, high-resolution, non-invasive imaging technique, that is proving to be valuable in the diagnosis of different skin conditions. In this study, we aimed to analyze the most common indications for LC-OCT in pediatric age group, discussing its potential role in clinical setting. METHODS: A retrospective review of the medical charts of patients ≤18 years of age, who were performed clinical, dermoscopy and LC-OCT for equivocal skin lesions, was conducted. Diagnostic confidence level was calculated for clinical/dermoscopic diagnosis alone and for combined clinical/dermoscopy and LC-OCT findings, based on a three-point scale ranging from 0% to 100%. RESULTS: Seventy-four skin lesions in 73 patients [(39 (53.4%) females and 34 (46.6%) males, mean age 13.2 (range 5-18 years) years] were investigated with LC-OCT. Diagnosis was established with histopathology in 23/74 (31.1%) cases, while 51/74 (68.9%) skin lesions were monitored over time or treated with topical/physical therapy. High diagnostic confidence increased by 21.6% after LC-OCT assessment, meanwhile reducing low and average score. CONCLUSIONS: LC-OCT may add practical clues for the identification of common skin conditions in pediatric population, improving diagnostic confidence and consequent tailored approach.

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 presen

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Background: Localization of atopic dermatitis (AD) in exposed areas such as the hands, head, and neck has been considered as a negative factor impacting on dupilumab response, although a comparison of exposed versus unexposed areas is not currently available. Objectives: The aim of this study is to evaluate the clinical response to dupilumab depending on the presence or persistency of AD skin manifestations in specific body areas. Methods: The study retrospectively collected clinical and demographic data of adult patients affected by moderate to severe AD. Based on the anatomical sites involved, 5 subcohorts of patients were identified. Results: A total of 41 patients were included in the study. Disease amelioration was detected during the study period, although baseline head/neck and hand localization was associated with a significantly lower likelihood of achieving an Eczema Area Severity Index (EASI) ≤1. In addition, patients with head/neck persistency showed a significantly lower response when compared to patients without persistency of head/neck AD in terms of both mean EASI and Dermatology Life Quality Index (DLQI) reduction. Conclusion: AD localization in exposed areas at the baseline and AD persistency at the head/neck may have a negative impact on certain treatment response parameters to dupilumab therapy.

The exposure to cold can induce the development of wheals and angioedema in a group of susceptible individuals: this phenomenon is largely known as cold-induced urticaria. The pathogenesis of cold-induced urticaria is not yet understood, although both autoallergens and immunoglobulin (Ig)E-mediated autoimmunity are presumed to be involved. Flares of cold-induced urticaria might depend on the release of histamine and other pro-inflammatory mediators, such as Interleukin (IL)-1, which is the predominating stakeholder of cryopyrin-associated periodic syndrome, a genetic disease characterized by cold-induced skin manifestations, including urticaria-like rashes. The majority of occurrence of cold-induced urticaria in children is idiopathic, but forms secondary to systemic conditions have been also reported. Primarily, the diagnosis remains a clinical process based on the history of patient, cold stimulation tests, and a few laboratory results, which could be useful for excluding any underlying disorders. The general rules to manage cold-induced urticaria in children can be summarized with cold avoidance, treatment with nonsedating antihistamines, and the anti-IgE monoclonal antibody omalizumab in selected patients. Familiar forms of cryopyrin-associated periodic syndrome could be prevented even in pediatric patients from the selective IL-1 blockade. Injectable epinephrine must be immediately used to manage the potential life-threatening manifestations occurring in a minority of children with cold-induced urticaria.

Introduction: Atopic dermatitis (AD) is a chronic inflammatory skin disease mediated by multiple signals including janus kinase (JAK)/signal transducer and activator of transcription (STAT) pathway. Current therapeutic armamentarium consists of a limited number of drugs which may result in the insufficient management of AD. Preclinical evidence regarding inhibition of JAK/STAT led to the development of a promising class of therapeutics, namely, JAK inhibitors. Baricitinib, a novel JAK1/JAK2 inhibitor is currently under investigation in AD clinical trials. Areas covered: This review offers an overview of Baricitinib and examines clinical efficacy and safety data in patients with moderate-to-severe AD. Expert opinion: Baricitinib showed promising preliminary data in terms of efficacy in phase II and III trials, with a very rapid onset of response and great improvements of itch and sleep disturbances. These aforementioned aspects combined with the advantage of an oral formulation have reduced drug production costs compared to biologic agents and could lead to consideration of baricitinib as a first line systemic treatment. Also, in some countries, it could be a therapeutic option in the case of contraindication or failure of conventional systemic drugs prior to biologic therapies. Data related to long-term safety and efficacy will be important to refine the place-in-therapy of this drug.

Ultrasonography has proven useful for diagnosis and treatment monitoring in patients with hidradenitis sup-purativa. The aim of this study was to assess the clinical response to adalimumab using ultrasound findings. This prospective study collected data on demographic features, disease severity, and hidradenitis suppura-tiva findings from patients with hidradenitis suppura-tiva treated with adalimumab. Generalized estimating equations investigated relationships between disease severity measures and clinical/demographic variables. The study included a total of 41 patients with hidra-denitis suppurativa who were treated with adalimu-mab for a mean ± SD period of 50.8 ± 32.2 weeks; range 6–108 weeks). Clinical improvement was observed during adalimumab therapy, with a progressively greater number of patients achieving HiSCR50 response (36.4% at week 52). Disease duration was identified as the most relevant clinical variable affecting disease severity and treatment response. Treatment response was also influenced by treatment duration, with a 4% greater likelihood of achieving HiSCR50 response at each time-point. At the ultrasound examination, sub-cutaneous involvement of hidradenitis suppurativa lesions was identified as a predictive negative factor for clinical response to adalimumab (HiSCR50 achie-vement).

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This is the first case of a pediatric mesalazine-induced erythema multiforme (EM), occurring in a teenager with Henoch-Schönlein purpura-related pancolitis. Our report highlights the wellknown contribution of salicylates to the development of severe skin reactions and also shows the importance of a pharmacological medical history in every case of EM.

Ist dies der erste Fall eines pädiatrischen Mesalazin-induzierten Erythema Multiforme (EM) bei einem Teenager mit Purpura Schönlein-Henoch-bedingter Pankolitis. Unser Bericht unterstreicht die bekannte Beteiligung von Salicylaten bei der Entstehung schwerwiegender Hautreaktionen und veranschaulicht darüber hinaus die Bedeutung der pharmakologischen Anamnese bei jedem Fall eines EM.

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