Epidemiology and geographic clustering of Erdheim-Chester disease in Italy and France.

This geoepidemiological study, performed in Italy and France, shows that Erdheim-Chester disease is increasingly diagnosed and cases cluster in specific geographic areas, namely southern Italy and central France. Disease frequency inversely correlates with the Human Development Index.

Analysis of current data on the use of topical mTOR inhibitors in the treatment of facial angiofibromas in tuberous sclerosis complex-An update.

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome causing hamartomatous growths in multiple organs. Facial angiofibromas occur in up to 80% of patients and can be highly disfiguring. Treatment for these lesions is challenging. Recently, topical rapamycin has been proposed as an effective option to treat angiofibromas but a commercially available compound has not yet been developed in Europe. We conducted a retrospective review with the aim to update the current data on the use of topical rapamycin in the treatment of angiofibromas in TSC, focusing on the optimal concentration and trying to establish which vehicle should be preferred. Thirty-nine reports describing the use of topical rapamycin in the treatment of angiofibromas in TSC were considered, involving a total of 483 patients. An improvement of the lesions has been shown in over 90% of subjects, particularly if the treatment was started at early stages. Several different formulations (ointment, gel, solution and cream) with a wide range of concentrations (0.003%-1%) were proposed, of which a pharmacological analysis has also been performed. Topical rapamycin can be considered an effective and safe option for the treatment and the prevention of facial angiofibromas in younger patients, but the best formulation has yet to be established. Our review demonstrates that ointment and gel should be preferred, but it is not clear which concentration is optimal. However, according to this study, the 0.1% concentration represents the first choice. Long-term and comparative studies between topical rapamycin formulations are required in order to establish which treatment has a better outcome and lower recurrence rate.

Ideal Features of Topical Antibiotic Therapy for the Treatment of Impetigo: An Italian Expert Consensus Report.

Background: 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. Objective: 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. Methods: 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. Results: 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. Conclusions: 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).

Epidemiology and clinical evolution of non-multisystem inflammatory syndrome (MIS-C) dermatological lesions in pediatric patients affected by SARS-CoV-2 infection: A systematic review of the literature.

COVID-19 can present with a range of skin manifestations, some of which specific of the pediatric age. The aim of this systematic literature review was to determine the type, prevalence, time of onset, and evolution of cutaneous manifestations associated with COVID-19 in newborns, children, and adolescents, after excluding multisystem inflammatory syndrome in children (MIS-C). PubMed, Tripdatabase, ClinicalTrials, and Cochrane Library databases were searched using an ad hoc string for case reports/series and observational studies, published between December 2019 and February 2022. Study quality was assessed using the STROBE and CARE tools. Seventy-three (49 case reports/series and 24 studies) out of 26,545 identified articles were included in the analysis. Dermatological lesions were highly heterogeneous for clinical presentation, time of onset, and association with other COVID-19 manifestations. Overall, they mainly affected the acral portions, and typically presented a favorable outcome. Pseudo-chilblains were the most common. CONCLUSIONS: Mucocutaneous manifestations could be the only/predominant and early manifestation of COVID-19 that could precede other more severe manifestations by days or weeks. Therefore, physicians of all disciplines should be familiar with them. WHAT IS KNOWN: • A variety of cutaneous manifestations have been reported in association with COVID-19. • Urticaria, maculopapular, or vesicular rashes can occur at any age, while chilblains and erythema multiforme are more common in children and young patients. WHAT IS NEW: • Skin lesions related to SARS-CoV-2 infection often show a peculiar acral distribution. • Mucocutaneous lesions of various type may be the only/predominant manifestation of COVID-19; they could present in paucisymptomatic and severely ill patients and occur at different stages of the disease.

Psoriasis and Cardiovascular Risk in Children: The Usefulness of Carotid Intima-Media Thickness.

Psoriasis is a skin disorder which mostly affects adults, beginning in childhood in almost one-third of patients. In adults it is associated with increased risk for cardiovascular diseases (CVD), while this association is still debated at younger age. Our aim was to evaluate the association between psoriasis and metabolic markers and cardiovascular findings in this age group. Twenty consecutive patients previously diagnosed with psoriasis (group A) were enrolled and compared with healthy non- psoriatic age- and sex-matched subjects (group B). The severity of the disease, CV risk factors, including anthropometric data with adiposity and its distribution, blood pressure (BP), laboratory metabolic tests, echocardiography and vascular ultrasound (transcranial echo-Doppler and carotid artery echo-Doppler with carotid intima-media thickness, cIMT) were performed for each subject. Personal history for CV risk, BP, anthropometric data were similar between the two groups, while familiar history for psoriasis was more frequent in group A (p < 0.02). C-IMT was significantly higher in group A compared to B (right, p = 0.001; left, p = 0.002). In addition, c-IMT was positively correlated with disease duration, triglycerides and triglycerides/glucose. Cerebral flow velocities, cardiac measurements, systo-diastolic function, ventricle geometry and mass were normal and comparable between the two groups, and did not correlate with CV risk factors. In childhood psoriasis c-IMT could represent a marker of pre-clinical cardiovascular involvement and contribute to start a personalized management, while cardiac findings seem to be normal in the early stage of disease. Longitudinal studies can clarify the progression of CV involvement in paediatric-onset psoriasis.

The Burden of Autosomal Recessive Congenital Ichthyoses on Patients and their Families: An Italian Multicentre Study.

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.

Viral rashes mimicking drug reaction with eosinophilia and systemic symptoms syndrome in children after ß-lactams intake: a diagnostic challenge.

In cases of skin eruptions over the course of antibiotic therapy and concomitant viral infection, differential diagnosis is often challenging. Confirming or ruling out drug hypersensitivity is not always a clear-cut question. Drug reaction with eosinophilia and systemic symptoms (DRESS) cases, for example, is classified as severe cutaneous adverse reactions due to drugs, but frequently the clinical manifestations do not completely fit into the diagnosis of DRESS. The aim of the present paper is to highlight similarities and differences among DRESS syndrome and DRESS-like rashes during viral infections and amoxicillin intake in children, in order to highlight those aspects that can help clinicians in early detection. We describe the dermatological, clinical, and laboratory characteristics of five patients hospitalized for DRESS-like skin rashes appearing roughly 1 week since the start of an amoxicillina course for upper respiratory tract infection (URTI) symptoms. The data are compared with those of 3 patients with early-onset DRESS syndrome. The absence of eosinophilia might be an initial marker to help identifying DRESS-like rashes; a quick clinical improvement and the confirmation of a viral infection able to explain the symptoms can help to finally rule out DRESS syndrome. Conclusion: A rapid, correct diagnosis of such DRESS-like rashes during viral infections allows more appropriate management and avoids unnecessary, life-long exclusion of useful and effective antibiotics because of a falsely "amoxicillin-allergy" labelling. What is Known: • Viral infections are common causes of skin rashes in children during antibiotic intake and may require differential diagnosis with drug reactions. • Early-onset DRESS syndrome is usually induced by antibiotics and appears ≤15 days after drug intake. What is New: • Prominent midface edema, maculopapular rash, and mild-to-moderate systemic symptoms may appear in children during viral illnesses treated with amoxicillin few days after drug intake, and may require differential diagnosis with early-onset DRESS. • In such cases, absence of eosinophilia, low (2-3) RegiSCAR score, confirmation of viral etiology, and a rapid resolution of the rash (2-5 days) might help to rule out DRESS; conversely, at an early stage, the presence of eosinophilia should suggest a diagnosis of DRESS.

Head and neck dermatitis, a subtype of atopic dermatitis induced by Malassezia spp: Clinical aspects and treatment outcomes in adolescent and adult patients.

BACKGROUND AND OBJECTIVES: Head and neck dermatitis (HND) is a clinical variant of atopic dermatitis (AD), presenting in adolescence or adulthood and characterized by involvement of the head, neck, and superior part of the trunk. The role of Malassezia spp has been advocated in the pathogenesis of HND, and antifungal agents represent the treatment of choice. METHODS: A retrospective single-center study was performed to define the clinical features and treatment response of HND among adolescent and adult patients. RESULTS: Thirty-one patients were identified, 17 with "adolescent-onset" and 14 with "adult-onset" HND. Adolescent-onset HND positively correlated with a past history of AD and presented with exclusive head and neck involvement (P < .05). Adult-onset HND was associated with concomitant widespread atopic eczema, involving the flexural areas of the upper and lower limbs, trunk, nipples, or hands (P < .05). A positive response to itraconazole in combination with topical treatments was observed in both groups. CONCLUSIONS: This study delineates two HND clinical phenotypes: adolescent vs adult onset. Different characteristics were observed in terms of relationship to AD and eczema localization. A history of AD in childhood and presentation with exclusive involvement of head and neck regions was observed predominantly in the adolescent-onset form, while adult-onset HND often occurred in association with diffuse dermatitis and a past history of AD was less frequent than in the adolescent group. The study is limited by the single-center retrospective nature, which may lead to diagnostic and selection biases, and the small cohort of patients.

Nevocentric erythema multiforme in a child.

A 9-year-old girl presented with multiple, ring-shaped, erythematous, nonscaly plaques on the trunk, face and arms, most surrounding preexisting melanocytic nevi. She had experienced recurring episodes of herpes simplex labialis over several years (average 4/year), the last occurring 10 days prior.

Vulvar vitiligo and lichen sclerosus in children: A clinical challenge.

Vulvar vitiligo (VV) and vulvar lichen sclerosus (VLS), both feature skin and mucosal hypo-/depigmentation. The aim of this study was to describe the clinical and dermoscopic features of VV and VLS in the pediatric population, providing diagnostic clues, and to define their association. We performed a systematic literature review of the clinical and dermoscopic features of pediatric VV and VLS. An observational study was conducted on children affected by VLS associated with VV, referred to the Dermatology Unit of the Sant'Orsola Polyclinic in Bologna, Italy. Medical history, age at diagnosis, ethnicity, clinical and dermoscopic features, and symptoms were recorded for all patients. 124 cases of VLS and 10 cases of VV were reviewed. Clinical manifestations included hypo-/depigmented patches in both conditions, while ecchymosis/purpura and fissures/erosion were observed in VLS. Symptoms including pruritus, pain, or burning were reported only by VLS patients. In our study five patients with VLS associated with VV were retrieved. Clinical features included well-demarcated depigmented patches in VV and translucent areas, erythema, ecchymoses/purpura, and labial fusion in VLS. Dermoscopy showed white structureless areas with a whipped cream-like appearance, linear or dotted vessels, white chrysalis-like structures, erosion and red-purpuric blotches in VLS and reduced pigment network or pigment absence, intralesional spots of residual pigmentation and telangiectasias in VV. Symptoms were present in all patients. Both VV and VLS show hypo-/depigmented patches. In the presence of associated symptoms, possible VLS should be investigated with clinical and dermoscopic examination to achieve a prompt diagnosis.

Tinea corporis incognito due to Microsporum Gypseum: Report of eight cases in children.

Microsporum gypseum is a geophilic saprophyte for which soil is the natural habitat. Tinea corporis caused by Microsporum gypseum is rare and usually occurs after direct contact with the soil or healthy carrier animals. We present a case series of eight children with tinea corporis caused by M gypseum, with unusual clinic presentations mimicking dermatitis.

Lichen sclerosus with enlarged vessels: A variant of lichen sclerosus in young girls.

Vascular findings have rarely been described in the setting of lichen sclerosus. Enlarged vessels within the atrophic plaques have been observed on the free margins of the labia minora and clitoral hood. The enlarged vessels completely remit upon treatment of lichen sclerosus with an ultra-potent corticosteroid ointment in the acute phase. During maintenance therapy with calcineurin inhibitors, there was no recurrence of the enlarged vessels.

Congenital fibrous hamartoma of the tip of the tongue: A novel peculiar entity.

Congenital fibrous hamartoma of the tip of the tongue, a peculiar and novel entity, consists of one or two asymptomatic pearly or yellowish nodules, not exceeding 0.5 cm in maximum diameter, at the tip of the tongue, ventrally or dorsally. Unlike other localizations, congenital fibrous hamartoma of the tip of the tongue is not associated with cleft lip or palate, or with feeding problems. Surgical excision should be avoided, reserved only for dubious cases, since the lesions are benign and stable over time.

Bei CALME (childhood asymmetry labium majus enlargement): die Ruhe bewahren und nachverfolgen.

CALME steht für childhood asymmetry labium majus enlargement und bezeichnet eine physiologische, nichtneoplastische Schwellung des Weichteilgewebes der Labia majora. CALME wird durch die Vergrößerung einer oder mehrerer normaler vulvärer Gewebeanteile verursacht und weist keine äußere Kapsel auf. Vereinzelte Berichte in der Literatur haben das Augenmerk auf diese gutartige Veränderung gelenkt und die Notwendigkeit betont, invasive Eingriffe mit möglichen Dauerfolgen zu vermeiden. Die Ätiopathogenese von CALME ist nach wie vor umstritten, wobei die Hypothese einer hormonell bedingten stromalen Hyperplasie der großen Schamlippen gegenüber der Annahme eines gutartigen neoplastischen Prozesses favorisiert wird. Dieser Artikel zielt darauf ab, einen detaillierten Überblick über die klinischen, histologischen und apparativen Befunde bei CALME zu geben und einen abgestuften Algorithmus zur Herangehensweise und zum Management von genito-inguinalen Geschwülsten bei pädiatrischen Patienten zu erstellen.

Keep CALME (childhood asymmetry labium majus enlargement) and follow up.

CALME is the abbreviation of "childhood asymmetry labium majus enlargement" and denotes a physiological, non-neoplastic swelling of labium majus soft tissues. It is caused by the expansion of one or more normal vulvar tissue components and is devoid of a peripheral capsule. A few reports in the literature have raised awareness of this benign condition and stressed the need to avoid invasive procedures with possible permanent sequelae. The etiopathogenesis of CALME is still debated, although the hypothesis of a hormone-driven major labia stromal hyperplasia is favored over the assumption of a benign neoplastic process. This article aims to provide a detailed review of the clinical, histological, and instrumental findings of CALME and provide a step-based algorithm to approach and manage genito-inguinal masses in pediatric patients.

Contact dermatitis due to masks and respirators during COVID-19 pandemic: What we should know and what we should do.

Given the current lack of a therapeutic vaccine for coronavirus disease 2019 (COVID-19), preventive measures including mask wearing are crucial in slowing the transmission of cases. However, prolonged wearing of protective respirators, medical and fabric masks can easily generate excessive sweating, moisture and friction. Closed and warm environments heighten the skin's permeability and sensitivity to physical or chemical irritants, leading to chronic cumulative irritant contact dermatitis or, rarely, even allergic contact dermatitis. Although not representing a life-threatening condition, contact dermatitis can have a significant impact on emergency management, as it is potentially able to reduce work performance and create emotional discomfort due to the involvement of evident body areas. To minimize the skin breakdown, adherence to standards on wearing protective and safe equipments and avoidance of overprotection should be performed. At the same time, some measures of skin care are recommended. Here, we offer some tips on how to prevent and manage contact dermatitis due to masks not only in health care workers, but also in the general population during this COVID-19 outbreak.

Cutaneous Infantile Haemangiomas with Intracranial and Intraspinal Involvement: A European Multicentre Experience and Review.

Infantile haemangiomas are very common benign tumours in the first months of life. They are mostly cutaneous; however, extracutaneous lesions are possible, and occur in very rare cases in the central nervous system. A European multicentre observational retrospective study was conducted in the last 5 years. Seven patients with intracranial or intraspinal infantile haemangiomas were selected and treated with oral propranolol. Propranolol was interrupted after complete or almost complete resolution of infantile haemangiomas. All patients tolerated the treatment well without side-effects. Central nervous system infantile haemangiomas are probably underestimated due to the frequent absence of symptoms and their spontaneous involution. However, they should be investigated in case of segmental cutaneous infantile haemangiomas, particularly on the head, neck, upper trunk, lumbar or sacral area in order to diagnosis intra-central nervous system involvement at an early stage.

Melorheostosis and Osteopoikilosis Clinical and Molecular Description of an Italian Case Series.

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.

Plaque-like myofibroblastic tumor, a rare entity of childhood: Possible pitfalls in differential diagnosis.

Plaque-like myofibroblastic tumor is a rare and benign pediatric soft tissue tumor. It presents as a slowly growing plaque reaching several centimeters in diameter, made up of multiple nodules. The clinical and histological features of this benign entity are similar to other fibrohistiocytic or myofibroblastic tumors occurring in childhood, so the diagnosis can be difficult. The correlation between clinical data, histopathology, and immunohistochemistry is necessary for the correct diagnosis.

Adalimumab in severe plaque psoriasis of childhood: A multi-center, retrospective real-life study up to 52 weeks observation.

The objective of this study is to determine drug effectiveness and safety of the tumor necrosis factor-alpha blocker monoclonal antibody adalimumab in a real-life cohort of 54 children and/or adolescents with severe plaque psoriasis. Retrospective, multicenter analysis over a 52-week period is discussed in this study. Efficacy was determined by the percentage of patients achieving Psoriasis Area Severity Index (PASI 75) and PASI 90 at weeks 16, 24, and 52 and the response in biologic-naïve versus non-naïve patients. Safety was assessed by the number of patients experiencing at least one adverse event. At week 16, 29.6% of patients achieved a 90% PASI score reduction (PASI 90), while 55.5% of patients achieved a 75% PASI score reduction (PASI 75). Effectiveness was sustained through week 24, since PASI 90 response increased to 55.5% and PASI 75 response increased to 74.0% of patients. The PASI response rates did not differ between biologic-naïve and non-naïve patients. The drug was well tolerated and no serious infections were observed. Adalimumab was effective and safe in this cohort of children with severe plaque psoriasis in a 52-week observation. Effectiveness did not differ between biologic-naïve and non-naïve patients.