Agminated Spitzoid Naevi after Remission of Langerhans Cell Histiocytosis: First Italian Case and Literature Review.

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Angioma serpiginosum: a case report and review of the literature

Abstract Background Angioma serpiginosum is a rare vascular anomaly whose pathogenesis is still unknown. It is characterized by the onset of vascular reddish macules and papules during childhood, lesions are usually monolateral with a linear serpiginous pattern. It is rarely associated with extracutaneous findings. This entity has not yet been included in the classification of the International Society for the Study of Vascular Anomalies. Case presentation We describe the first Italian report of angioma serpiginosum with a congenital symmetrical presentation. The patient had a further extension of macules during puberty involving both of the soles. No extracutaneous manifestations were present. Diagnosis was confirmed with dermoscopy and light microscopy that revealed the typical clusters of dilated, thickened and PAS+ capillaries in the upper dermis. Moreover, Immunohistochemistry showed positive WT-1 staining. Genetic analysis with next generation sequencing did not detected any mutation. Conclusions Our patient presented a peculiar symmetrical and planar extension with a serpiginous linear pattern. The proliferative nature of this condition has been widely discussed in literature. In our case immunohistochemistry was positive for Wilms tumor-1, a new endothelial marker expressed during angiogenesis in reparative processes and endothelial tumors. Clinical evolution, histological and immunohistochemical findings suggest that angioma serpiginosum should be considered as a vascular proliferation. For these reasons we think it should be included in the international classification as a tumor

EGFR inhibition for metastasized cutaneous squamous cell carcinoma in dystrophic epidermolysis bullosa

Dystrophic epidermolysis bullosa (DEB) is a hereditary skin fragility disorder, characterized by trauma-induced blistering followed by soft tissue fibrosis. One of the most feared complications is the early development of aggressive cutaneous squamous cell carcinomas (SCC). For patients with locally advanced or metastasized SCCs treatment with cetuximab, a monoclonal antibody against epidermal growth factor receptor (EGFR), has been proposed and so far, treatment of five DEB patients with cetuximab has been published. With this report, we extend the spectrum of EB patients treated with cetuximab by adding two additional patients. Taking together all DEB cases treated with cetuximab, we propose that cetuximab should be administered as early as possible, since it seems to be more efficient and is accompanied by rather mild adverse effects. We also show that EGFR is frequently expressed in DEB-associated SCCs, although there were noticeable differences in the level of expression, which may influence responsiveness to EGFR-targeting therapies. Although only limited experiences with targeted cancer treatments in EB exist, such reports highlight the treatments' effects in this specific cohort and assist our therapeutic decisions

Mental health during the COVID-19 pandemic and first lockdown in Lebanon: Risk factors and daily life difficulties in a multiple-crises setting.

IntroductionResearch from around the world shows important differences in the impact of the COVID-19 pandemic and lockdowns on mental health. This study examined the extent of mental health challenges (depressive and anxiety symptoms and daily life difficulties) and their associations with pandemic- and response-related factors during the first lockdown in Lebanon, which happened amid a severe economic crisis and socio-political turmoil.MethodsData come from a cross-sectional internet-based survey (May-June 2020). Association of depressive (Patient Health Questionnaire (PHQ-9)) and anxiety symptoms (Generalized Anxiety Disorder (GAD-7)) with outbreak-related worries and knowledge, information sources, and confidence and satisfaction in response measures were estimated using logistic regression adjusted for sociodemographic and socioeconomic indicators.ResultsAmong 510 participants (mean age 36.1±11.3; 69.4% women), 32.3% had elevated depressive and 27.3% had elevated anxiety symptoms; younger age, unemployment, loss of employment, and lower income were related to more mental health symptoms. Most prevalent daily life challenges were feelings of uncertainty (74.5%) and financial (52.2%) and emotional (42.2%) difficulties; these and all other daily life difficulties (work-related, caregiving, and online learning) were significantly higher among participants with higher depressive and anxiety symptoms. Higher outbreak-related worries were associated with higher depressive (OR = 1.36, 95% CI = 1.20,1.53) and anxiety symptoms (OR = 1.47, 95% CI = 1.30,1.67). Higher pandemic-related knowledge, reliance on and trust in local health agencies and professionals' information, and satisfaction and confidence regarding governmental and health institutions' response were all related to lower mental health symptoms.ConclusionResults show that mental health burden in Lebanon during the first lockdown (when the COVID-19 outbreak was still minimal) is among the highest reported worldwide and highlight elevated emotional and financial tolls and widespread impact on daily life. In this high-burden and multiple-challenges context, results suggest an important role for the healthcare body, knowledge, and trust in the institutions managing the response

Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations

Abstract Background Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement. Case presentation We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years. Conclusions Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder

Paraneoplastic pemphigus and Castleman’s disease: a case report and a revision of the literature

Abstract Background In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman’s disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. Case presentation We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms. Conclusions Reviewing literature, 20 clinical cases have been described in the pediatric age. Diagnosis may be challenging, requiring an average of 3 months (range from 3 weeks to 2 years). In all cases, the initial manifestations were mucocutaneous lesions, especially oral lesions with poor response to conventional treatment. Systemic symptoms may be present as well. Therapeutical approach is still discussed with no consensus. Almost all patients received corticosteroids with poor response. Other drugs including azathioprine, methotrexate, cyclosporine and monoclonal antibodies have been evaluated for the control of the disease. Further studies and experimental trials urge to define the diagnostic criteria and therapy protocol

The Influence of Brushing Motion on the Cutting Efficiency of Two Heat-Treated Endodontic Files: An In-Vitro Micro Computed Tomography Study

Background: To assess the cutting efficiency of two heat-treated endodontic files with a brushing motion in oval-shaped canals. Methods: A total of 10 intact lower molars with a single oval distal canal were selected and randomly divided into two groups according to the system used for shaping: 2Shape and Hyflex CM. The instrumentation was performed in two parts: a first shaping according to the manufacturer’s instructions and final shaping using 5 brushing motions on the distal, lingual, and buccal walls, avoiding the danger zones. Micro computed tomography (micro-CT) scans before and after the brushing motion were superimposed at all three levels: coronal, middle, and apical. Canal changes in buccolingual (ΔBL) and mesiodistal (ΔMD) dimensions were measured. Data were statistically analyzed by repeated-measures analyses of variance and the student t-test (p < 0.05). Results: For ΔMD, no significant difference emerged between the 2Shape and Hyflex CM in the apical and coronal thirds. However, the cutting efficiency was significantly greater with the 2Shape in the middle third (p < 0.05). Regarding ΔBL, no statistically significant difference was detected between 2Shape and Hyflex CM comparing the anatomical thirds, while 2Shape had a significant higher cutting efficiency considering the total BL diameter (p < 0.05). Conclusions: Under the limits of a vitro study, the shaping procedure with 2Shape and brushing motion was more efficient than the Hyflex CM in the midroot levels in terms of ΔMD, and in total canal space for ΔBL. Both files ensured an effective mechanical preparation

Update on the Management of Pediatric Psoriasis: An Italian Consensus

Introduction Psoriasis affects children with a considerable burden in early life. Treating pediatric psoriasis is challenging also because of the lack of updated specific guidelines. With the recent approval of several biologics for pediatric psoriasis and the ongoing COVID-19 pandemic, the management of young psoriatic patients is facing major changes. A revision of treatment recommendations is therefore needed. Methods In September 2021, a board of six Italian dermatologists convened to update treatment recommendations. The board issued evidence- and consensus-based statements covering relevant areas of pediatric psoriasis, namely: assessment of psoriasis severity, management of children with psoriasis, and treatment of pediatric psoriasis. To reach consensus, the statements were submitted to a panel of 24 experts in a Delphi process performed entirely via videoconference. A treatment algorithm was produced. Results There was full consensus that psoriasis severity is determined by the extension/severity of skin lesions, site of lesions, and impact on patient quality of life. Agreement was reached on the need for a multidisciplinary approach to pediatric psoriasis and the importance of patient/parents education. The relevance of vaccinations, including COVID-19 vaccination, for psoriatic children was acknowledged by all participants. Management issues that initially failed to reach consensus included the screening for psoriasis comorbidities and early treatment with biologics to prevent them and the use of telemedicine to facilitate patient follow-up. There was full consensus that topical corticosteroids are the first choice for the treatment of mild pediatric psoriasis, while phototherapy and systemic therapy are used in children with moderate-severe psoriasis. According to the proposed treatment algorithm, biologics are the first line of systemic therapy. Conclusions Targeted systemic therapies are changing the treatment of moderate-severe pediatric psoriasis, while topical corticosteroids continue to be the first choice for mild disease. Children-centered research is needed to further improve the treatment of pediatric psoriasis