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The natural evolution of Spitz-Reed nevi is poorly understood, owing to the lack of studies investigating their longterm clinical evolution and the fact that most of the lesions are surgically excised. The surgical approach is indicated because of the clinical and histopathologic similarities with melanoma that may create diagnostic confusion in some cases. Hence nowadays, due to the low incidence of melanoma in children and the development of diagnostic dermoscopic criteria, a less aggressive approach to SRN may be reserved, especially in cases with typical features. We report a case of a pigmented lesion that showed gradual changes, from a Spitz Reed Nevus with typical clinical and dermoscopic features, to a common nevus.

Bullous pemphigoid (BP) is an autoimmune bullous disease and is a rare condition in childhood. Acquired tense acral bullae and fixed urticarial annular lesions on the trunk are diagnostic clues of infantile BP. Diagnosis is supported by immunosorbent assay (IgG anti-BP180 and BP230) and direct immunofluorescence (linear deposition of IgG at the dermo-epidermal junction). Topical and/or systemic corticosteroids are the first-line treatment. The prognosis is good with a self-limited clinical course. Differential diagnoses include impetigo and other bullous diseases in children, such as dermatitis herpetiformis, linear IgA bullous dermatosis and erythema multiforme. The etiopathogenesis is still unknown, and the role of antigen stimuli such as infections, drugs and vaccination is still debated.

Inherited epidermolysis bullosa (EB) encompasses a group of rare genetic disorders of the epidermal and dermal-epidermal junction complex, characterized by fra- gility of the skin and the mucous membranes, causing blistering and erosions after minor injury. The different types of EB present extreme phenotype variability; while some are mild, chronic and compatible with life, others are severe and associated with complications, morbidity, and increased mortality. Skin and mucous membranes are primarily affected by EB, but extracutaneous organs may be involved. The main types of EB include EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler syndrome (KS).

Spitz nevi are benign melanocytic neoplasms characterized by epithelioid or spindle melanocytes or both. In some rare cases their presentation overlaps with the clinical and histopathologic features of malignant melanoma, so a differential diagnosis can be difficult to make. Intermediate forms between Spitz nevi and malignant melanoma, with unpredictable behavior, have been called atypical Spitz tumors. A literature search was performed to review the clinical, dermoscopic, genetic, and histopathologic aspects of spitzoid tumors. Spitz nevi mainly occur in children, with no predilection for sex, and in young women. Common sites are the head and lower arms, where Spitz nevi present as pink nodules or hyperpigmented plaques. Spitzoid lesions may have diverse dermoscopic patterns: vascular, starburst, globular, atypical, reticular, negative homogeneous, or targetoid. The management of spitzoid lesions can be invasive or conservative; surgical excision is usually reserved for those with doubtful features, whereas clinical and dermoscopic follow-up is preferred for typical pediatric Spitz nevi. The role of sentinel lymph node biopsy in atypical Spitz tumors is debated. Immunohistochemistry and new molecular techniques such as comparative genomic hybridization, polymerase chain reaction, and fluorescence in situ hybridization offer new diagnostic perspectives, investigating genetic alterations that are specific for malignant melanoma or for Spitz nevi.

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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 (FDA). The Italian Study Group for Cutaneous Annexial Disease of the Italian Society of dermatology (SIDeMaST) 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.

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.

We report here our experience on the use of dermoscopy for the detection of subungual red comets, which are sometimes present in the nails of patients affected by tuberous sclerosis complex. Dermoscopy allowed us to visualize, with better resolution than the naked eye, very tortuous capillaries surrounded by a whitish halo and close parallel binary tortuous capillaries. In some cases, subungual red comets are associated with the presence of periungual or subungual fibromas, but their exact pathogenesis remains unknown.

Nevus comedonicus is a rare, benign hamartoma of the pilosebaceous unit that may be isolated or part of the nevus comedonicus syndrome. Although rare in children, complication by hidradenitis suppurativa-like lesions has been described. We present a report of a 9-year-old girl in whom surgical excision was curative, with a review of the relevant literature.

Background Pyogenic granuloma (PG) is a common, acquired, benign vascular neoplasm of the skin and mucous membranes. It occurs most often in children and adolescents. First-line treatment options for PG are based on destructive approaches. Pain, permanent scarring, and pigmentation are potential complications of these therapies. Methods This single-arm, open-label, prospective study evaluated the efficacy of topical propranolol for treatment of PGs in children. All patients clinically diagnosed with cutaneous PG consecutively at the Dermatology Pediatric Outpatient Service of the University of Bologna from January 2010 to December 2010 received a compounded formulation of propranolol ointment 1%. Results We found propranolol ointment to be effective in consecutive patients; 59.0% completely regressed in a mean of 66 days, 18.2% remained stable, and 22.7% did not respond. No side effects (eg, skin irritation, allergy, bleeding) were observed. Conclusion Topical propranolol ointment 1% with occlusion appears to be an effective treatment for PGs in children. Early treatment was associated with a more favorable outcome.

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.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.

Background Erythema palmare hereditarium (EPH), also known as Lane's disease, is a rare, benign condition presenting as persistent erythema involving the palms. EPH can appear at birth or later in life and usually in at least two members of the same family, although a sporadic case has been reported. Methods We report five cases of EPH and offer a review of the current literature. The first and second cases are twin boys presenting with erythema mainly on the thenar and hypothenar eminences and on the phalanges that appeared 8 months after birth. The third case is a girl with congenital palmar erythema and two other capillary malformations. The fourth case is a 58-year-old woman with palmar erythema that appeared after pregnancy. Her 32-year-old daughter had presented with the same palm redness since birth. Results A review of the literature shows that women are affected almost three times more than men. Dermoscopic evaluation showed red structureless areas with arborizing vessels, mainly running parallel along follicular openings. Conclusion EPH should be considered in all patients presenting with palmar erythema, especially in familial long-lasting forms. It can be congenital or acquired, but the pathogenetic mechanism is unclear. To the best of our knowledge, this is the first dermatoscopic study of EPH and the largest case series reported in the literature, involving two families and one sporadic case.

An 8-month-old Caucasian infant with neurofibromatosis type 1 presented with a congenital plexiform neurofibroma and multiple café au lait spots. A pale area surrounded one of the café au lait spots located on the left gluteus in the area of dermal melanocytosis. This halolike phenomenon results from the disappearance of the Mongolian spot around the café au lait spots, revealing normal pigmented skin. This sign has been described rarely in the literature and the pathogenic mechanism is unclear.

neonatal and infantile skin diseases are frequently encountered in the clinical practice and represent worldwide a socioeconomic issue. They encompass a wide range of acquired or congenital conditions, including infections, vascular lesions and inflammatory diseases and can present with different degrees of severity, leading in some cases to dramatic complications. Areas covered: In this paper we report the most recent evidences on the management of some common skin diseases in neonates and infants. Hemangiomas, viral, fungal and bacterial infections, omphalitis, atopic and seborrhoeic dermatitis, napkin disease will be treated and discussed. Expert opinion: The majority of the dermatologic alterations in neonates are physiological, transient and do not require any treatment, thus the parents can be reassured about the good prognosis. However, in some cases, serious conditions must be excluded. In particular neonatal and infantile infections should be promptly recognized and properly managed, to avoid severe complications. The therapeutic options include traditional and, although few, innovative medical treatments, which will be carefully taken into consideration by the expert Dermatologists and Paediatricians.

Background Melanoma is an infrequent neoplasm in children and adolescents. The number of consultancies for melanocytic nevi control in the pediatric population is increased by the major anxiety of parents and pediatricians. Methods A retrospective chart review was performed in order to evaluate the incidence rate of melanoma, Spitz nevi, congenital and acquired melanocytic nevi in patients aged 14 years or less and referred to our institution from April 2010 to September 2015. Results A total of 32,755 dermatologic pediatric consultancies were performed in the considered 66 months period, including 4260 patients referred for mole control (13%). During the same period, a total of 5193 excisions of melanocytic lesions were performed (adults and pediatric patients), 259 of which were performed in 250 patients aged 14 years or less (4.98%). Only 1 in situ melanoma of the nail matrix was detected in a 12-year-old female patient (0.38% of surgical excisions). The number needed to excise (NNE) in our pediatric population, calculated by comparing the total number of excised lesions and the number of melanomas found, was 259. Conclusions Our data leads to 3 major observations: 1) the rarity of melanoma in patients with 14 years of age or less is confirmed; 2) the number of excisions performed in this pediatric population is extremely high; 3) neither the cases of melanoma and atypical Spitz neoplasm (ASN) nor the majority of Spitz nevi were referred to us for a generic mole control. Mole controls in the pediatric population should be properly addressed, incrementing the attention on specific suspicious cutaneous lesions.

Skin disinfection with chlorhexidine gluconate has not been standardized in preterm infants. We present 5 cases of chemical burns that occurred within the first 2 days of life in very low birth weight neonates after skin disinfection with aqueous and alcohol-based chlorhexidine solutions.

Key Clinical Message The presence of history of redness and swelling of toe in an infant without history of trauma is typical of hair threat tourniquet syndrome. The treatment simply involves incision and removal of hair fibers. Physicians should be aware of this syndrome because early diagnosis and treatment avoid serious complications.

From January 2010 to December 2012, 296 skin biopsies were performed in pediatric patients using only local anesthesia (cream and infiltration). The biopsies were divided into three groups: biopsies of skin neoplasms, biopsies of skin rashes and biopsies of follicular-centered lesions. Our data demonstrate the possibility of using this procedure, with the dual advantage of eliminating hospitalization and cost savings.

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This mono-center randomized, controlled, double-blind study evaluates the safety and efficacy of MD2011001 cream versus placebo, in mild-to-moderate atopic dermatitis (AD). MD2011001 is a nonsteroidal topical cream containing vitamin E, epigallocatechin gallate and grape seed procyanidins.Patients with AD (corresponding to an IGA score of 2 or 3), involving the face, the perioral/periocular area and/or the neck, were enrolled. Patients were randomized 1:1 ratio to receive MD2011001 or placebo before the start of the study (D0), then evaluated after 7 days, and after 28 days. The study was approved by the Local Independent Ethics Committee and conducted according to the Declaration of Helsinki and local regulations. The statistical tests used were the Wilcoxon test and the Mann-Whitney U-test.Forty-four patients (29F and 15M) were enrolled. The IGA values showed a statistically significant reduction during the treatment period obtaining a favorable safety profile and local tolerance for both the products. The reduction in the surface area affected by AD was significantly faster with MD2011001.This study focuses on very sensitive areas known to be particularly susceptible to local complications.These results suggest the usefulness of an emollient treatment for mild/moderate AD.

Background Atypical forms of hand, foot, and mouth disease (HFMD) caused by coxsackievirus A6 have been reported in recent years. High fever and severe cutaneous lesions are common, whereas neurologic complications are rare. Eczematous areas of patients with atopic dermatitis show more lesions. The goal of the current study was to describe the clinical characteristics of children with atypical HFMD and to investigate the involvement of the different enterovirus serotypes associated. Methods All patients referred to our service for atypical HFMD from January 2012 to February 2014 were enrolled and classified as having the diffuse form (lesions extended to the trunk), the acral form (lesions with a mainly acral distribution), or eczema coxsackium (lesions on preexisting eczematous areas). Results Data from 47 patients were analyzed (median age 22 months [range 4–84 mos]); viral genotyping was performed in 11 cases. Sixty-two percent of the subjects developed the acral form, 23% eczema coxsackium, and 15% the diffuse form. Most patients had a nonclassical vesicular eruption and moderate to severe extent of cutaneous involvement. Approximately 80% of patients had palmoplantar purpuric macules. Most children younger than 2 years old had the acral form, most patients with eczema coxsackium were age 2 years and older, and the diffuse form was similarly distributed between the two age groups. Coxsackievirus A6 was detected in 9 of 11 genotyped cases. Conclusion Our prospective study allowed the identification of three HFMD phenotypes differing from the classical form. Clinical care of these patients should include symptomatic treatment of extracutaneous features and, if necessary, hospitalization for complications.

A 3-year-old boy presented with a 4-day history of fever and diffuse itchy eruption, spreading from the face. His medical history was unremarkable, except for a recent diagnosis of adenoidal hypertrophy treated with oral betamethasone sodium phosphate for 10 days followed by budesonide nebuliser. Physical examination showed erythematous patches with mild scaling of the face and, on the forearms, hands and legs, fixed hive-like target lesions (figure 1), with …

Autosomal recessive congenital ichthyosis (ARCI) is a clinically and genetically heterogeneous group of genodermatoses characterized by disorders of cornification. ARCI11 (MIM #602400) includes two syndromes caused by mutations in the ST14 (suppression of tumorigenicity 14) gene: IFAH (autosomal recessive congenital ichthyosis, follicular atrophoderma and hypotrichosis) and ARIH (autosomal recessive ichthyosis and hypotrichosis). All the reported ARCI11 cases so far belonged to consanguineous Turkish or Arab families]. We describe a novel p.Glu519Gln missense mutation in a patient affected by IFAH from a non consanguineous Rumanian family.

A 7-day-old girl, born at term and otherwise healthy, was referred with multiple congenital skin lesions on her right arm. Physical examination showed multiple vascular slightly reticulate patches with deep purple rim and a blue-to-greyish atrophic centre on the right forearm (figure 1). Figure 1 Vascular slightly reticulate patches with deep purple rim and …

Bullous pemphigoid is rare in children and even rarer in infants. By presenting two cases of bullous pemphigoid related by their temporal proximity with a previous episode of vaccination, it will be carefully discussed if a relationship is or is not possible. Our final conclusion is that the association is mostly a myth rather than a reality and bullous pemphigoid is not a contraindication to continue with the normal vaccination schedule of infants. It is important to know about this clinical entity in order to perform adequate treatment that avoids any worsening or future relapse of this disease.

Purpose of the Study: Our aim was to perform an extensive search of the literature on the prevalence of nail psoriasis in the pediatric population and to estimate it on our own database. Procedures: Searching the most important databases yielded results of 16 papers published in a 40-year period which reported the prevalence of nail psoriasis in children. Furthermore, data gathered between 2004 and 2013 at two centers specialized in nail disorders were analyzed. Results: The selected papers encompassed a total of 4,853 psoriatic children, of whom 762 (15.7%) had nail involvement. Concerning our data, a total of 68,839 children were seen in pediatric skin consultation in both clinics over a period of 10 years, 0.11% of which had nail involvement. The rate of psoriatic children presenting nail alterations was 19.4% in the Greek and 15.5% in the Italian patient groups. Conclusions and Message: This is the retrospective study including the largest number of cases of nail psoriasis in the pediatric population. Our data are in line with the incidence estimation of the rest of Europe.

A 14-month-old child presented with a 4-day history of diaper rash consisting of round erosions with a thin crust on an erythematous background symmetrically distributed around the anal orifice and crops of perilesional vesicles (figure 1). During the examination, further lesions were identified on the extremities and hand-foot-mouth disease (HFMD) caused by coxsackie virus A6 (CVA6) was confirmed on serology. Figure 1 (A) …

Journal of Pediatrics, The - In Press.Proof corrected by the author Available online since lundi 5 mai 2014

A one-month-old infant was admitted to the paediatric unit with high fever (38.5°C) and irritability. He was otherwise healthy and born at term. His parents reported contact with a cousin with high fever. The physical examination was unremarkable except for tense fontanelle. Suspecting infection, an antibiotic (amoxicillin) and antiviral (ganciclovir) therapies were started. However, the blood count, the C-reactive protein and the cerebrospinal fluid analysis were normal, …

Background Nail dystrophies in newborns are rare and are particularly frightening when they appear as masses involving a single digit, suggesting the possibility of a tumor. Objective The aim of this study is to report 3 infants with a congenital pseudoclubbing appearance of a digit and with a reddish discoloration caused by the presence of a subungual hemangioma. Methods Diagnosis was based on clinical examination and ultrasonography. Results Clinical presentation and vitrocompression showed the presence of a vascular mass. Ultrasonography confirmed the diagnosis of a subungual hemangioma. The lesions showed a spontaneous regression on follow-up. Conclusion Localization of hemangioma under the proximal nailfold is extremely rare and produces nail pseudoclubbing caused by capillary vessel proliferation in the soft tissue of the subungual region, which is associated with a reddish discoloration of the nail that typically fades with compression. Hemangiomas of infancy located in the proximal nailfold are rare, and, in our opinion, not at risk to become big masses with tissue damage and compression.

A 20-day-old, otherwise healthy female presented with a 2-week history of small perianal ulcers. These lesions appeared on the fourth day of life as some discrete ulcers overlying an area of macular erythema on the right buttock. The ulcers enlarged slowly, despite two courses of topical antibiotics for impetigo. She was the product of a full-term pregnancy. Birth weight was 3870 g. Physical examination showed multiple perianal ulcerations coalescing …

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

The Italian Consensus Conference on clinical management of atopic dermatitis in children reflects the best and most recent scientific evidence, with the aim to provide specialists with a useful tool for managing this common, but complex clinical condition. Thanks to the contribution of experts in the field and members of the Italian Society of Pediatric Allergology and Immunology (SIAIP) and the Italian Society of Pediatric Dermatology (SIDerP), this Consensus statement integrates the basic principles of the most recent guidelines for the management of atopic dermatitis to facilitate a practical approach to the disease. The therapeutical approach should be adapted to the clinical severity and requires a tailored strategy to ensure good compliance by children and their parents. In this Consensus, levels and models of intervention are also enriched by the Italian experience to facilitate a practical approach to the disease.