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Background/Purpose: Phototherapy has emerged as a safe yet effective form of treatment of atopic dermatitis (AD). Few studies have been done to evaluate the efficacy of phototherapy in Asian children. The aim of this study was to review the phototherapy experience in a cohort of Asian pediatric patients with AD at a tertiary dermatologic center in Singapore. Methods: A retrospective study of patients 18 years and below with AD who had undergone phototherapy at KK Women's and Children's Hospital, Singapore, over a 4‐year period was performed. Results: Sixty‐two patients were identified, between ages 4 and 16 years (mean age 11 years) at the time of commencement of phototherapy. Thirty‐five (60%) patients were males and 23 (40%) were females. Most patients had moderate to severe disease, with 60.3% of the patients with an initial body surface area (BSA) involvement of 31%–60% and 13.8% of the patients with an initial BSA involvement of 61%–90%. For patients who had undergone narrowband ultraviolet B (NBUVB) and combined ultraviolet A (UVA) and NBUVB phototherapy, the mean reduction of the Eczema Area and Severity Index (EASI) scores were 11.4 and 7.9, respectively. Common side effects experienced include xerosis, pruritus, erythema, and pain. Other reasons for cessation of therapy in the NBUVB group included time commitment difficulty (9.3%), hyperactivity (2.3%), and claustrophobia (2.3%). Two patients that had photochemotherapy (psoralen + UVA) [PUVA] suffered from post‐UVA burns requiring cessation of treatment. More than half of the patients (56.9%) treated with phototherapy experienced treatment success with improvement in Investigator Global Assessment and EASI scores. 86.2% of the patients had good compliance to the treatment regime, 12% had poor‐compliance, and 3.4% were lost to follow‐up. Conclusion: Phototherapy is a useful treatment adjunct for moderate to severe AD in Asian children. [ABSTRACT FROM AUTHOR]

Simple Summary: Neurofibromatosis Type 1 is an inherited tumour predisposition syndrome with a varied clinical phenotype. Long-term monitoring through imaging is inconsistent and varies in high- and low-income countries. Implementation of a clinical practice guideline through a multidisciplinary clinic is instrumental to the care of adult Neurofibromatosis Type 1 patients. This systematic review aims to evaluate the association between a country's socioeconomic status and diagnostic modalities and strategies used for adult Neurofibromatosis Type 1 patients. Our results show multiple imaging modalities are used in high-income countries; however, there is limited use in low-income countries. The two most common diagnostic modalities used in developed countries are WB MRI and FDG PET/CT. Background: Neurofibromatosis Type 1 is an autosomal dominant tumour-predisposition condition commonly diagnosed in childhood and fully penetrant by adulthood. Long-term monitoring through imaging is inconsistent and varies between high- and low-income countries. Implementation of a clinical practice guideline through a multidisciplinary clinic is instrumental to the care of adult Neurofibromatosis Type 1 patients. We aim to systematically review international diagnostic modalities and strategies to evaluate any association between a country's socioeconomic status and diagnostic modalities or strategies used for Neurofibromatosis Type 1 patients. Methods: We searched PubMed, Embase, Web of Science, and Cochrane. Relevant clinical information on the surveillance of adult Neurofibromatosis Type 1 patients worldwide was reviewed, extracted, and synthesised. Results: We identified 51 papers reporting on 7724 individuals. Multiple imaging modalities are actively employed in high-income and upper-middle-income countries for surveying adult Neurofibromatosis Type 1 patients. We did not find any relevant papers from low- and middle-income countries. Conclusions: This systematic review suggests that there is robust data on diagnostic modalities for adult Neurofibromatosis Type 1 patients in high-income countries, but not for low- and middle-income countries. There is a lack of data on consolidated diagnostic strategies from both high- and low-income countries. Efforts should be made to publish data on usual clinical practice in low- and middle-income countries to develop clinical practice guidelines describing best medical practice to fit a local context. [ABSTRACT FROM AUTHOR]

In this work, a lightweight compliant glove that detects scratching using data from microtubular stretchable sensors on each finger and an inertial measurement unit (IMU) on the palm through a machine learning model is presented: the SensorIsed Glove for Monitoring Atopic Dermatitis (SIGMA). SIGMA provides the user and clinicians with a quantifiable way of assaying scratch as a proxy to itch. With the quantitative information detailing scratching frequency and duration, the clinicians would be able to better classify the severity of itch and scratching caused by atopic dermatitis (AD) more objectively to optimise treatment for the patients, as opposed to the current subjective methods of assessments that are currently in use in hospitals and research settings. The validation data demonstrated an accuracy of 83% of the scratch prediction algorithm, while a separate 30 min validation trial had an accuracy of 99% in a controlled environment. In a pilot study with children (n = 6), SIGMA accurately detected 94.4% of scratching when the glove was donned. We believe that this simple device will empower dermatologists to more effectively measure and quantify itching and scratching in AD, and guide personalised treatment decisions. [ABSTRACT FROM AUTHOR]

Background: Inherited epidermolysis bullosa (EB) is a group of genodermatoses with considerable clinical and genetic heterogeneity. Clinical diagnosis of the EB subtypes is frequently imprecise and requires confirmation with genetic testing. There is still limited study using genetic testing to identify EB subtypes in Indonesia. This study aims to identify the pathogenic variants of inherited EB patients at the Department of Dermatology and Venereology, Universitas Padjadjaran—Dr Hasan Sadikin General Hospital in Bandung, West Java, Indonesia and to describe the correlation between the phenotype and genotype of our patients. Methods: Twelve patients clinically diagnosed with EB were included in this study. Genetic testing was performed in collaboration with KK Women's and Children's Hospital, Singapore. Results: Pathogenic variants were identified in the COL7A1 gene in seven patients, namely Dominant Dystrophic EB (DDEB) with mutation types c.5945G>T, c.6218G>A, Recessive Dystrophic EB (RDEB) c.2005C>T, c.6081dup, c.1268C>T, c.1784C>T which are all known mutations. Novel mutations were found in the COL7A1 gene in two patients namely DDEB c.6253G>T and RDEB c.6740C>T. Two EB Simplex (EBS) patients showed mutation KRT14 gene as c.356T>C, c.373C>T which are known mutation. In addition, a novel mutation in LAMA3 gene c.2649del was found in one Junctional EB (JEB) patient. Conclusion: The molecular diagnoses of 12 Indonesian EB patients were identified, of which three were novel pathogenic variants. Concordance between the initial clinical diagnosis and genetic testing was only 33%. This demonstrated the importance of early genetic testing for accurate diagnosis, prognostication, management and genetic counselling. [ABSTRACT FROM AUTHOR]

The article discusses a case of epidermolytic epidermal nevus on the genitalia caused by a mosaic KRT10 mutation in a 17-year-old male. The patient presented with verrucous skin lesions over the foreskin, shaft, scrotum, and perineum, with histopathologic features consistent with epidermolytic hyperkeratosis. The mutation was found to be somatic and not present in the germline DNA, indicating a de novo occurrence. The article highlights the importance of genetic testing in cases of epidermal nevi with features of epidermolytic hyperkeratosis for accurate diagnosis and counseling on potential transmission risks. [Extracted from the article]

This article discusses the case of a 6-year-old girl with persistent exfoliative dermatitis. The girl presented with painful rashes on her neck, chest, axillary, and abdominal region, along with abdominal pain. Despite treatment for eczema, her condition did not improve. Further investigation revealed that she had a deficiency in vitamin B3 (niacin), leading to a diagnosis of pellagra. Treatment with oral niacinamide resulted in complete resolution of the rash and abdominal pain. Pellagra is a condition that was historically characterized by dermatitis, diarrhea, dementia, and death, but is now rare in developed countries due to fortification of flour with vitamin B3. However, it is still endemic in certain areas due to poor nutrition. [Extracted from the article]

Identification of mutations in I LAMB3 i indicates that both had JEB, the rarest form of EB.4 For generalized EB caused by mutations in I LAMB3 i which account for 80% of all laminin-322 mutations, having at least one missense mutation tends to be associated with the less severe intermediate non-Herlitz type. Dear Editor, Epidermolysis bullosa (EB) is a group of heterogeneous disorders involving fragility of epithelial tissues. [Extracted from the article]

Atopic dermatitis (AD) is a chronic inflammatory disease associated with pruritus and sleep loss. Pine-tar has long been used for various chronic skin conditions in which its polycyclic aromatic hydrocarbon (PAH) component is anti-inflammatory and its resin acids antiseptic. The null hypothesis of this trial is that there is no difference in clinical efficacy between a pine-tar product and its vehicle for AD. A 3-month, investigator-blinded, crossover, randomized control trial (RCT) was conducted in which each patient was assigned to bathing with pine-tar bath oil for one month and vehicle bath oil for another, with a washout period of 1-month in-between. Acceptability and efficacy of the bath products were measured. Disease severity scores (scoring atopic dermatitis (SCORAD) and patient-oriented eczema measure (POEM), quality of life questionnaires, noninvasive skin biophysiological measurements, blood IgE levels, and Staphylococcus aureus (SA) colonization status were assessed before and following bathing. Significant improvements were found in total SCORAD (p =.030), POEM (p =.004), SA colonization status (p =.002), and log-transformed IgE level (p =.009) among patients who bathed with pine-tar in the overall RCT study using intention-to-treat analysis. For per protocol analysis, significant improvements were found in total SCORAD (p =.024), objective SCORAD (p =.011), extent (p =.014), intensity (p =.032), pruritus (p =.047), POEM (p =.044), SA colonization status (p =.035), and log-transformed IgE level (p =.028). Acceptability to both bath-oils was good, and no product-related serious adverse events were recorded. Bathing with pine-tar is an efficacious and recommendable adjuvant practice for AD patients. Disease improvement is associated with reduction of SA and IgE. [ABSTRACT FROM AUTHOR]

Juvenile xanthogranuloma (JXG) is the most common non‐Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of literature on JXG in Asian children. We aim to describe the epidemiology, clinical features, systemic associations, histological features and outcome of a cohort of Asian children with JXG, and review the literature on the condition. We retrospectively reviewed the demographic, clinical and histological data of patients less than 16 years of age, diagnosed with JXG at our tertiary pediatric hospital between January 2002 and April 2019. A total of 147 children with JXG were identified, with a slight male preponderance of 53.1%. The median age of the onset was 15.5 months, with 69.4% presenting before 2 years of age. There was no racial predilection. The most frequently involved site was the head and neck region (44.2%). The majority of patients (76.2%) presented with a solitary lesion. Spontaneous resolution was documented in 57.7% of our patients with mean duration to resolution of 18.8 months. The proportion and speed of resolution did not differ in children with single or multiple lesions. No ophthalmologic complications were detected in our study cohort. JXG in children is generally limited to the skin and is rarely associated with systemic involvement, including the eye. Unless clinically indicated, the results from our study does not support routine screening for juvenile myelomonocytic leukemia, eye or systemic complications, even in the setting of multiple cutaneous JXGs. [ABSTRACT FROM AUTHOR]

Background: Pediatric allergic contact dermatitis (ACD) is increasingly prevalent. Patch testing is the gold‐standard diagnostic investigation. The aims of our study were to describe the clinical profile of pediatric patients with ACD in a multi‐ethnic Asian population and identify the common contact allergens. Methods: This was a retrospective study involving children and adolescents aged 16 years or younger with clinically suspected ACD who underwent patch testing between January 2007 and March 2020 at two institutions in Singapore. Information pertaining to their demographics, atopy history, clinical presentation, and patch test results was analyzed. Results: A total of 252 patients were included. The mean age was 10.9 years (1.0–16.7 years). The majority were Chinese (79.8%) and girls (57.5%). Many (66.3%) had atopic dermatitis (AD), which was mild. The most common presentation was an acral eczematous rash. The sensitization rate was 50.0%. The most frequent reactions were to nickel sulfate (49.2%) and fragrance mix (19.1%). The overall rate of relevant positive patch tests was 72.5%. Patients with AD were less likely to have a reaction to fragrance mix (p =.019) and more likely to have a reaction to disperse blue (p =.041). Compared to younger children, adolescents were less likely to have a positive patch test (p =.008). Indians were also less likely to have a positive reaction (p =.004). Conclusion: Metals and fragrances were the most common allergens causing childhood ACD in Singapore. Patients with AD were less likely to be sensitized to fragrances and more likely to be sensitized to disperse blue dye. [ABSTRACT FROM AUTHOR]

Background: CHILD syndrome is an X‐linked dominant disorder associated with pathogenic mutations in the NSDHL gene. The condition is predominantly found in females as it is lethal in males. Most cases present at birth with extensive unilateral ichthyosiform erythroderma involving the trunk and limbs. Milder and less extensive presentations have been reported, leading to misdiagnosis especially during early childhood. Methods and Results: We report an adult female of Malay ancestry who presented with minimal skin and limb involvement. She was only diagnosed in adulthood when she presented with gastrointestinal symptoms and worsening of skin manifestations. The clinical diagnosis was suspected after a combination of clinical, pathological and immunohistochemistry correlation, and molecularly confirmed with the discovery of a frameshift variant in NSDHL. The novel variant was inherited from her mother who had some linear hypopigmented patches over the medial aspects of both her arms and right forearm. Conclusion: We uncovered a novel frameshift variant associated with presentations that cast a new light on the clinical features of CHILD syndrome. [ABSTRACT FROM AUTHOR]

Adenoviruses are common viral pathogens in childhood; however, cutaneous manifestations are not well‐documented. We present a rare case of cutaneous adenovirus infection in a 23‐month‐old boy with a background of CD40 ligand deficiency, post bone marrow transplant. The clinical morphology of the skin lesions in our patient, described as skin‐colored papules with central crusting, has not been previously described and contributes to the growing literature of cutaneous adenovirus cases. [ABSTRACT FROM AUTHOR]

Background: Atopic dermatitis (AD) is a common, chronic dermatosis, with onset of disease often manifesting in early infancy. Past studies evaluating the early use of moisturisers in the prevention of AD had mixed results. Objectives: To compare the incidence of moderate or severe AD and total incidence of AD in a cohort of 'at‐risk' infants treated with moisturisers from the first 2 weeks of life, to a similar group without moisturisers. Methods: We performed a single‐centre, prospective, parallel‐group, randomised study in infants with at least 2 first‐degree relatives with atopy. Subjects were randomised into either a treatment group with moisturisers or a control group without moisturisers. Participants were assessed at 2, 6, and 12 months for AD and if present, the severity was assessed using SCORAD index. We also compared the overall incidence of AD, trans‐epidermal water loss (TEWL), stratum corneum (SC) hydration, pH, and incidence of food and environmental sensitisation and allergies between both groups. Genotyping for loss‐of‐functions mutations in the FLG gene was conducted. Results: A total of 200 subjects were recruited, with 100 subjects in each arm. There was no significant difference in incidence of moderate or severe AD, and total incidence of AD at 12 months between the treatment and control groups. There was a lower mean SCORAD in the treatment group than in the control group, but no significant difference in TEWL, SC hydration, and skin pH. No significant side‐effects were reported. Conclusions: The early use of moisturisers in 'at‐risk' infants does not reduce the incidence of moderate‐to‐severe AD and overall incidence of AD in infancy. [ABSTRACT FROM AUTHOR]

We report a case of junctional epidermolysis bullosa with pyloric atresia (JEB‐PA) with minimal skin involvement but severe protein‐losing enteropathy and airway involvement. Genetic analysis revealed heterozygous mutations in the ITGB4 gene encoding integrin β4 protein. Parental testing confirmed inheritance of frameshift variant (c.794dupC) as maternal and splice site variant (c.1608C>T/p.Cys536Cys) as paternal. Immunofluorescence mapping of her skin revealed a subepidermal blister with decreased and frayed integrin β4 at both the floor and the roof of the blister, while the intestinal mucosa showed complete absence of integrin β4. We review the literature and discuss the differential expression of integrins in the skin and gastrointestinal tract, as well as the role of chronic inflammation in the pathogenesis of EB. [ABSTRACT FROM AUTHOR]

Atopic dermatitis (AD) is a common, chronic, recurrent inflammatory skin disease. Poorly controlled AD can lead to reduced quality of life (QoL) and psychosocial impairment. Dupilumab is the first approved monoclonal antibody targeting type 2 inflammation, for adolescent and adult patients with moderate‐to‐severe AD. We performed a retrospective analysis of the efficacy and safety of dupilumab in a cohort of Asian children and adolescents with moderate‐to‐severe AD. Clinical response was documented with investigator global assessment (IGA) and eczema area and severity index (EASI) scores. Improvement in QoL was assessed using child dermatology life quality index (CDLQI) or Teenager's quality of life (T‐QoL), and caregivers' QoL was assessed using dermatitis family impact (DFI) questionnaire. Twelve patients were recruited, aged between 6 to 18 years of age (mean 13.3 years), with mean duration of AD of 9.8 years. At baseline, the mean IGA score was four and the mean EASI was 48.2. The mean T‐QoL and DFI scores at baseline were 18.7 and 19.6, respectively. After 12 to 16 weeks of treatment, the mean IGA score decreased to 2.2. The mean EASI decreased to 19.3 with mean reduction of 28.9. The mean T‐QoL decreased to 7.5 with mean reduction of 11.2, and the mean DFI decreased to 8.6 with mean reduction of 11. Adverse events included mild conjunctivitis in two patients and paradoxical head and neck erythema in one patient. Our study supports dupilumab as an effective and safe treatment option for Asian children and adolescents with moderate‐to‐severe AD. [ABSTRACT FROM AUTHOR]

Cellular neurothekeoma is a rare, benign cutaneous neoplasm. We report a case of cellular neurothekeoma arising on the mid‐back of a 10‐year‐old boy within the previous radiation therapy field for medulloblastoma. Clinical features of cellular neurothekeoma in children are similar to those in adults, except that there have been no reports of local recurrence in children following surgical excision, and the presence of atypical histological features does not predict clinical behavior. Recognition of this entity in children is important to avoid misdiagnosis and overly aggressive treatments. [ABSTRACT FROM AUTHOR]

Background: Acute generalized exanthematous pustulosis (AGEP) is a rare, severe, cutaneous adverse reaction. Although most commonly caused by drugs, it can also be triggered by infections, especially in children. Methods: This is a retrospective study involving children and adolescents aged 16 years or younger, diagnosed with AGEP between January 2010 and March 2020 in our tertiary pediatric hospital. Information pertaining to the patient's demographics, clinical presentation and progress, biochemical, microbiological, and histopathological investigations, treatment, and outcomes was analyzed. Results: Eight patients were diagnosed with AGEP with mean age 8.2 years (range: 1.7‐16.0 years). None of the patients had a personal or family history of psoriasis. Almost all patients had fever (n = 7, 87.5%). Although all 8 patients had intercurrent illness, 5 cases were attributed to infection, while the other 3 were likely precipitated by drugs. Abnormal hematological and biochemical parameters included a raised absolute neutrophil count (mean: 11.5 × 109/L, range: 5.0‐30.9 × 109/L), C‐reactive protein (mean: 52.5 mg/L, range: 5.0‐143.7 mg/L), and erythrocyte sedimentation rate (mean: 38.6 mm/h, range: 6‐64 mm/h). All patients developed post‐pustular desquamation and subsequently recovered. The mean duration from onset to cessation of acute pustulation was 5.6 days (range: 3.0‐10.0 days). One patient developed a recurrent episode of AGEP. Conclusion: AGEP is rare and may be more commonly caused by infections in children. The condition is self‐limiting with overall good outcomes in this age‐group. In cases with concomitant infection and drug use, formal allergy testing should be arranged after resolution of the infection to confirm the underlying etiology. [ABSTRACT FROM AUTHOR]

Introduction: Infantile haemangiomas (IH) are the most common vascular tumours in childhood. Over the past decade, treatment of IH has been revolutionised by the discovery of the effectiveness of beta-blockers in its treatment. We review our hospital's experience with oral propranolol in the treatment of IH in an Asian population.Methods: We performed a retrospective review of the medical records and clinical photos of paediatric patients with IH treated with propranolol in a tertiary paediatric hospital in Singapore from January 2010 to February 2015.Results: A total of 88 patients with IH treated with propranolol were identified over a five-year period, with 79 patients included in the final analysis. There was a predominance of female patients (75.9%) and preterm infants (41.8%) in our study population. The head and neck (65.8%), especially the orbital or preorbital region (45.6%), were the most common lesion sites in our cohort of patients. Mean age of onset was 2.3 ± 4.5 weeks of age, and mean age of starting propranolol treatment was 7.7 ± 10.5 weeks of age. 44.3% of patients experienced > 75% improvement, while 29.1% experienced improvement of 50%-75%. Response to treatment was influenced by the age of starting treatment.Conclusion: Our study provides further evidence of the efficacy and safety of propranolol in the treatment of IH in an Asian population. Early treatment is recommended for optimal results. [ABSTRACT FROM AUTHOR]

Objective: To test the utility and diagnostic yield of a medical-exome gene panel for identifying pathogenic variants in Mendelian disorders.Methods: Next-generation sequencing was performed with the TruSight One gene panel (targeting 4813 genes) followed by MiSeq sequencing on 216 patients who presented with suspected genetic disorders as assessed by their attending physicians.Results: There were 56 pathogenic and 36 likely pathogenic variants across 57 genes identified in 87 patients. Causal mutations were more likely to be truncating and from patients with a prior clinical diagnosis. Another 18 promising variants need further evaluation for more evidence to meet the requirement for potential upgrade to pathogenic. Forty-five of the 92 clinically significant variants were novel.Conclusion: The 40.3% positive yield compares favourably with similar studies using either this panel or whole exome sequencing, demonstrating that large gene panels could be a good alternative to whole exome sequencing for quick genetic confirmation of Mendelian disorders. [ABSTRACT FROM AUTHOR]

Viral warts or verruca are very common skin infections in children. Although benign, lesions can be extensive, painful, bleed, or lead to cosmetic disfigurement. Although spontaneous resolution can occur, parents often bring their children for treatment, especially when they are symptomatic. Many publications have assessed the efficacy and safety of treatment of warts in adults. However, treatment in children can be challenging due to their immune responses and lower threshold for pain. We review the current literature on the methods, efficacy, and side effect profile of common treatment modalities for cutaneous viral warts in children. There is evidence that salicylic acid and cryotherapy are effective, and although cryotherapy is more effective, there is a higher risk of side effects such as pain and blistering. Combination treatment with salicylic acid and cryotherapy may reduce these side effects. Although there is limited data, other treatment options such as cantharidin, immunotherapy and other mechanical therapies, for example, carbon dioxide lasers, may also be considered, especially for recalcitrant lesions. [ABSTRACT FROM AUTHOR]

Introduction: Moisturizers are one of the mainstays of the topical treatment of atopic dermatitis (AD). One of the adverse effects of moisturizers is skin irritation, especially on excoriated AD skin. We compared the potential for irritation of two commercially available moisturizer products for the treatment of AD: a ceramide-based moisturizer (Ceradan® Cream; Hyphens Pharma Pte Ltd, Singapore) and a urea 5% moisturizer (Aqurea Lite Cream; ICA Pharma Pte Ltd, Singapore). Methods: We performed a prospective single-blind randomized controlled study recruiting AD patients aged between 8 and 16 years with symmetrical or near symmetrical scratch marks (excoriations) of at least grade 2 to 3 severity score, according to the Eczema Area and Severity Index (EASI), over bilateral antecubital fossae. Subjects were randomized to receive the ceramide-based moisturizer to either the left or right antecubital fossa or urea 5% cream to the other antecubital fossa. Subjects were asked to grade the immediate skin irritation of both creams on a standard visual analogue scale (VAS) and which cream they would prefer to use as a daily moisturizer. Primary outcome was the mean irritant score of each cream, and secondary outcome was the subjects' preference of either cream as their daily moisturizer. Results: A total of 42 participants were enrolled with a mean age of 11 years 5 months. The ceramide-based cream had a significantly lower mean VAS score (mean 0.69, SD = 1.63) for irritation compared with urea 5% cream (1.43, SD = 1.64) (p = 0.035). More participants also preferred the ceramide-based cream over urea 5% cream (62% versus 38%) as their daily moisturizer, but this did not reach statistical significance (p = 0.164). Conclusions: A ceramide-based moisturizer may be considered as a suitable choice for children to minimize irritation from moisturizer treatment for AD. [ABSTRACT FROM AUTHOR]

Background/objectives: Atopic dermatitis is associated with a decreased health‐related quality of life and contributes to substantial health care costs. It is important to understand what accelerates health care costs to inform various stakeholders, so they can effectively meet health care needs. This cross‐sectional study aims to explore associations between health‐related quality of life, health care costs, and cost‐accelerating variables. Methods: Information on health‐related quality of life (HRQoL) was collected through generic and disease‐specific instruments from caregivers and children <16 years of age with a physician‐confirmed diagnosis of atopic dermatitis. The economic impact of atopic dermatitis was evaluated by analyzing information on health service utilization and other health care costs related to managing the condition. Results: Children with high impact on health‐related quality of life presented an annual health care cost of US$ 3787 compared with US$ 2548 for moderately impacted and US$ 2258 among children for which the condition had low impact. The severity of atopic dermatitis, disease duration, and a lower health‐related quality of life was associated with greater health care costs. Analyses of subdomains of health‐related quality of life revealed correlations between "mood" and "personal relationships" on one hand and caregivers' physical health and health care costs on the other hand. Conclusions: Highly affected HRQoL is associated with increased health care costs and could be a valuable complement to traditional severity assessments. By using HRQoL instruments, burdens and symptoms beyond severity can be identified and addressed with interventions to increase HRQoL and subsequently reduce health care costs. [ABSTRACT FROM AUTHOR]

Background: Atopic dermatitis (AD) is a common dermatosis. The cornerstone of eczema management is to repair and maintain skin barrier and hydration, as well as to reduce inflammation. Wet wrap therapy (WWT) is a widely used adjunct to achieve this. The conventional material used for WWT is viscose, which presents drawbacks including discomfort, high cost, and poor durability. Here, we explore the possibility of using customized nanotextile (nanopolyester) for WWT, hoping to prove that this material is non‐inferior to viscose in clinical effectiveness and patient acceptance. Methods: Patients aged 0‐18 years with moderate to severe eczema were randomized to receive either viscose (Tubifast™) or nanotextile for WWT. Patients were instructed to apply WWT daily overnight for 2 weeks. Patients' disease severity score (IGA, SCORAD) and quality of life (QoL) score (IDQOL/CDLQI) were measured on day 0, 7, and 14 of treatment. Patient survey was conducted to collect patients' feedback about garment use. Results: Fifty‐three children aged 7 months to 17 years were recruited (27 in Tubifast™ and 26 in nanotextile group). Patients in both groups showed significant improvement in disease severity and QoL from baseline (P <.001), and such improvement was similar in both groups. However, nanotextile garment was significantly more comfortable (2.73/10 vs 5.12/10, P =.001), easier to wear (2.78/10 vs 5.24/10, P =.003), and cooler (2.43/10 vs 3.96/10, P =.033) from patients' feedback. Conclusion: This study demonstrates that nanomaterial is as effective as conventional viscose in WWT, while superior in patient acceptability. Nanotextile for WWT has good potential in eczema management, especially in patients with suboptimal response to topicals alone. [ABSTRACT FROM AUTHOR]

CARD14‐associated papulosquamous eruption (CAPE) is a rare autosomal dominant dermatosis that presents classically in early childhood with clinical features of both psoriasis and pityriasis rubra pilaris (PRP). The disease is known to be refractory to topical and systemic therapies classically used for psoriasis, with the majority of reported cases requiring treatment with biologics, such as ustekinumab and secukinumab. We present a toddler with a clinical presentation consistent with CAPE and found to have a novel heterozygous variant of the CARD14 gene. She was refractory to treatment with topical emollients and topical corticosteroids, but responsive to oral acitretin. [ABSTRACT FROM AUTHOR]

Immunodeficiency secondary to anti-interferon-gamma (anti-IFN-γ) autoantibodies was first described in 2004 as an acquired defect in the IFN-γ pathway leading to susceptibility to multiple opportunistic infections, including dimorphic fungi, parasites, and bacteria, especially tuberculosis and non-tuberculous mycobacterium (NTM) species. It has so far only been described in adult patients. We present 2 cases of disseminated NTM infections in otherwise immunocompetent children. A 16-year-old girl with Sweet's syndrome–like neutrophilic dermatosis developed recurrent fever and cervical lymphadenitis secondary to Mycobacterium abscessus. A 10-year-old boy with a history of prolonged fever, aseptic meningitis, aortitis, and arteritis in multiple blood vessels developed thoracic vertebral osteomyelitis secondary to Mycobacterium avium complex. Both patients were found to have positive serum neutralizing anti-IFNγ autoantibodies. Testing for anti-IFNγ autoantibodies should be considered in otherwise healthy immunocompetent hosts with recurrent or disseminated NTM infection. This represents a phenocopy of primary immunodeficiency which has been recently described only in adults. We report the first two cases of this phenomenon to affect children. [ABSTRACT FROM AUTHOR]

Background: Atopic dermatitis (AD) is a common skin condition in childhood. AD management can be complex, and caregivers may have inadequate information on the disease, aggravating factors, and management. Comprehensive therapeutic education has been closely associated with increased compliance and is recommended for all patients. There are, as yet, no studies on the impact of a pharmacist‐led eczema counseling service. Objectives: The primary objective was to assess the impact of a pharmacist‐led eczema counseling service on improving caregivers' knowledge. The secondary objective was to evaluate caregivers' satisfaction of the service and their confidence in handling the patient's condition after counseling. Methods: This was a prospective, questionnaire‐based observational study. After obtaining informed consent, caregivers were required to complete questionnaire A, containing items on demographics and knowledge assessment. Caregivers then underwent a counseling session by a dermatology‐trained pharmacist. After counseling, caregivers were required to complete questionnaire B containing items on satisfaction and confidence level. A follow‐up phone call was conducted after initial counseling session, during which knowledge‐based questionnaire C would be completed. Results: Data from 32 participants were included in the analysis. There was a significant improvement in caregivers' knowledge score after receiving counseling, with mean precounseling score of 8.38 ± 3.92 and mean postcounseling score of 13.88 ± 3.65 (P < 0.001). Majority was satisfied with the service and was confident in handling their child's condition. Conclusion: Pharmacist‐led eczema counseling has a positive impact on caregiver's knowledge and can lead to improved compliance. The service was well received, although more time could be spent during the counseling. [ABSTRACT FROM AUTHOR]

Novel mutations and clinical outcomes of copper-histidine therapy in Menkes disease patients. A systematic review and evidence-based guideline for diagnosis and treatment of Menkes disease. Genetic analysis is now the gold standard for the diagnosis of the disease.9 Treatment of Menkes disease is with copper supplementation. [Extracted from the article]

Degos disease, also termed malignant atrophic papulosis, is a rare systemic vaso‐occlusive disorder, seldom reported in the pediatric population. The pathognomonic skin lesion in Degos disease is a papule with an atrophic porcelain‐white center with an erythematous, telangiectatic rim. The benign form of the disease remains limited to the skin, whereas, in others, it progresses to thrombotic vasculopathy in multiple organs including the gastrointestinal, cardiorespiratory, and central nervous systems, with a high mortality rate. We present a rare case of Degos disease in an adolescent female, presenting as acute renal failure secondary to thrombotic vasculopathy, with the characteristic skin lesion distinctively seen on dermoscopy. [ABSTRACT FROM AUTHOR]

Lipschütz ulcers are uncommon acute, painful genital ulcerations occurring in adolescent or young adult females, with rare reports in children. Behcet's disease is a chronic condition that presents with recurrent oral and genital ulcers, associated with systemic manifestations.[3] Pyoderma gangrenosum manifests as large, tender, chronic ulcers that rarely involve the vulva.[5] Histology is usually non-diagnostic. Six patients presented with a single ulcer and four patients with multiple ulcers varying between 0.5 and 2 centimeters in diameter (Figs. [Extracted from the article]

Atopic dermatitis (AD) is a common chronic, pruritic skin disease in children. As the incidence of AD increases, especially in high-income countries, paediatricians may see an increasing number of recalcitrant AD cases in their practice. Although these cases are principally managed by paediatric dermatologists, it is important for paediatricians to be aware of the use and side effects of non-topical treatment like phototherapy and systemic agents as well as the evidence for alternative treatment, which caregivers may ask about. This review presents paediatric evidence for the practical use of phototherapy and certain oral immunosuppressants for paediatric AD including doses, duration of use and monitoring of adverse effects. The use of alternative therapy including traditional medicine, probiotics and the role of nutrition are also discussed. Narrow band ultraviolet B phototherapy is effective in recalcitrant paediatric AD. When phototherapy is ineffective or contraindicated, systemic drugs may be administered cautiously with close surveillance of side effects. The use of azathioprine, ciclosporin, methotrexate and mycophenolate mofetil is generally safe in the short term under close monitoring and can be effective alongside compliance to topical treatment. Alternative complementary treatment is not known to be effective. Holistic management including therapeutic patient education is important. Good quality paediatric studies for non-topical AD treatment are needed for definitive guidelines. [ABSTRACT FROM AUTHOR]

Capillary malformation-arteriovenous malformation syndrome (CM-AVM) is an autosomal dominant condition, associated with heterozygous pathogenic variants in I RASA1 i and I EPHB4 i genes, characterized by multiple capillary malformations (CMs) with associated fast-flow vascular anomalies such as arteriovenous malformations (AVMs) and arteriovenous fistulas.1 Pulsed dye laser (PDL) is an established treatment for cutaneous slow-flow vascular lesions but its use has not been well reported for the treatment of the CMs in CM-AVM.2,3 We report a case of a young child with CM-AVM who responded well to treatment with PDL. Good response to pulsed dye laser in patients with capillary malformation-arteriovenous malformation syndrome (CM-AVM). Treatment of capillary malformations in capillary malformation-arteriovenous malformation syndrome with pulsed dye laser. [Extracted from the article]

Background Melioidosis, caused by Burkholderia pseudomallei, is endemic in Singapore and can present as localized or disseminated disease. Methods Demographic data, clinical features, investigation results, treatments, and outcomes in patients aged <16 years diagnosed with melioidosis at KK Women's and Children's Hospital between January 2002 and January 2014 were retrospectively reviewed. Data for patients with primary skin disease and those with other organ involvement were compared. Results Seventeen children were diagnosed with melioidosis. Their median age was 12.5 years (range: 2-15 years). Nine (53%) patients presented with localized cutaneous melioidosis and five (29%) with localized lymphadenitis, pneumonia, or septic arthritis. The remaining three (18%) patients had melioidosis sepsis; two of these patients died from septic shock. Treatment included an initial 1-2 weeks of IV antibiotics followed by 3-6 months of oral combination antibiotics. All cases of localized cutaneous disease resolved completely with no recurrences. Three (60%) of the five patients with localized involvement of other organ systems achieved complete resolution of disease, and the remaining two were lost from follow-up. Conclusions Although uncommon, melioidosis can occur in children living in endemic regions. Patients with localized skin disease have good outcomes with no recurrences. Systemic disease can be fatal, especially in the presence of underlying immunodeficiency. Diagnosis requires a high index of suspicion, and treatment requires prolonged combination antibiotic therapy. [ABSTRACT FROM AUTHOR]

Background Nontuberculous mycobacterium ( NTM) infections are rare in children, with limited published studies. The course of the disease can be variable and there are no accepted treatment guidelines for the management of NTM infections in children. Objective To review a cohort of pediatric patients admitted to a tertiary pediatric hospital in Singapore for cutaneous NTM infections. Methods A retrospective review was performed of all children admitted to KK Women's and Children's Hospital with cutaneous NTM infections from 2002 to 2012. Results Sixty-seven patients with positive NTM cultures from various body sites were identified. Eight of the 67 patients (11.9%) presented with cutaneous NTM without evidence of systemic involvement. The mean age at diagnosis for these eight patients was 10 years (range 5-21 yrs). Mycobacterium abscessus was the most common NTM isolated (five patients), followed by Mycobacterium hemophilium (two patients) and Mycobacterium kansasii (one patient). Most patients presented with isolated skin abscesses. Two patients were immunocompromised. Six patients required multidrug antibiotic treatment for a median duration of 5.5 months (range 3-17 mos). The median follow-up duration was 8.5 months (range 2 wks-29 mos). Conclusion Although the incidence of cutaneous NTM is rare, the diagnosis should be considered in patients presenting with chronic wounds. Most patients require treatment with multidrug antibiotic therapy, although uncomplicated abscesses can be treated with surgical incision and drainage alone. [ABSTRACT FROM AUTHOR]

Vitiligo is a common acquired progressive depigmenting condition that can have devastating psychological effects in dark-skinned patients. We performed a retrospective review of patients younger than 16 years of age with a clinical diagnosis of vitiligo treated using phototherapy at the National Skin Center, Singapore, over a 5-year period. Seventy-one Asian patients ages 5 to 15 years when they underwent phototherapy were identified. There was a higher proportion of Indian patients in our cohort than in the population. The duration of disease ranged from 2 months to 12 years. More than half of the patients had generalized vitiligo and more than one-third had segmental vitiligo. Patients with generalized vitiligo had a better response than those with segmental vitiligo. Reported response rates were highest for narrowband ultraviolet B ( UVB) phototherapy, followed by targeted phototherapy combining ultraviolet A1 ( UVA1) and UVB; 308-nm excimer lamp phototherapy and paint psoralen-UVA photochemotherapy had marginally lower reported response rates. The duration of treatment ranged from 3 to 40 months and the total number of treatments ranged from 20 to 209 sessions. Reported side effects were mild and included itching, scaling, erythema, pain, sunburn, blistering, and phototoxicity. We consider phototherapy to be a safe and efficacious modality for the treatment of vitiligo in Asian children. [ABSTRACT FROM AUTHOR]

Acne, herpes simplex virus, isotretinoin, retinoid herpeticum Diagnosis: retinoid herpeticum A swab from the affected skin for herpes simplex virus (HSV) polymerase chain reaction (PCR) was positive for HSV type 1 DNA. Keywords: acne; herpes simplex virus; isotretinoin; retinoid herpeticum EN acne herpes simplex virus isotretinoin retinoid herpeticum 1318 1319 2 11/11/21 20210901 NES 210901 CASE PRESENTATION A 14-year-old girl presented with facial rash and fever for 5 days. [Extracted from the article]

Vitiligo is an acquired depigmenting disorder affecting 0.5-2% of the population worldwide, and as many as half of these cases develop before the age of 20 years.1 There are few Asian studies characterising the epidemiology of paediatric patients with vitiligo. Topical therapies included topical corticosteroids (mometasone 0.1% furoate cream, betamethasone 0.025% valerate cream, desonide 0.05% cream) and topical calcineurin inhibitors (tacrolimus 0.1% ointment, tacrolimus 0.03% ointment, pimecrolimus 1% cream). Of the 72 patients with documented outcomes, there was good response in 9 patients (12.5%), partial response in 50 (69.4%) patients and poor response in 13 (18.1%) patients. [Extracted from the article]

A 14-year-old boy who had undergone a matched sibling bone marrow transplant for acute lymphoblastic leukemia presented with painful nodules on his palms after prolonged gaming on his computer and mobile phone. Histology showed a neutrophilic inflammatory infiltrate surrounding the acrosyringium and eccrine sweat coils in the deep dermis. The lesions resolved spontaneously with conservative management. [ABSTRACT FROM AUTHOR]

The goal of the current study was to review a cohort of Asian children with atopic dermatitis ( AD) treated using phototherapy at a tertiary dermatologic center in Singapore. We performed a retrospective review of patients younger than 16 years old with a clinical diagnosis of AD treated with phototherapy at the National Skin Center, Singapore, over a 5-year period from 2004 to 2008. Twenty-five patients were identified who were ages 7 to 15 years at the time they underwent phototherapy, with equal sex distribution. The duration of disease ranged from 2 to 14 years. Most patients had extensive disease involving more than 70% of their body surface area. Fifteen patients were treated with narrowband ultraviolet B ( NBUVB) phototherapy, with 10 (66%) showing improvement of symptoms. The duration of therapy was 3 to 30 months (mean 11 mos), with a mean of 60 treatment sessions (range 12-104). Nine patients were treated with combined ultraviolet A ( UVA) and NBUVB phototherapy. Five patients (55%) improved, with four having good response and one having mild improvement. The duration of therapy was 1-6 months (mean 3.6 mos), with an average of 22 treatment sessions (range 10-44). Two patients underwent combined UVA/broad-band UVB ( BBUVB) phototherapy. Both had worsening of disease requiring cessation of treatment. Phototherapy is a useful adjunct in the treatment of moderate to severe AD in children. More than half of the patients treated with NBUVB or combined UVA and NBUVB improved with phototherapy, but the usefulness of combined UVA and BBUVB requires further evaluation. [ABSTRACT FROM AUTHOR]

Few studies have evaluated Asian children with mycosis fungoides ( MF). We report a series of patients from a tertiary dermatologic institution in Singapore. A retrospective review was performed of patients younger than 16 years old diagnosed with MF between 2000 and 2008 at the National Skin Centre, Singapore. Forty-six patients were identified. At initial presentation, a provisional diagnosis of MF was made in 19 patients (41.3%), pityriasis lichenoides chronica ( PLC) in 11 (23.9%) and postinflammatory hypopigmentation due to eczema or other causes in 11 (23.9%). After skin biopsy, the hypopigmented variant of MF was diagnosed in 42 patients (91.3%). There was one case each of PLC-like MF, pigmented purpuric dermatosis-like MF, classic MF, and solitary MF. Pityriasis lichenoides coexisted in three cases (6.5%). All except one patient presented with the early patch-plaque stage of disease (stage IA/ B). The disease did not progress in any of our patients after a mean follow-up of 71.0 ± 52.5 months. Twenty-seven patients (58.7%) had complete disease clearance after a mean duration of 27.1 ± 28.1 months; 15 (49.7%) of 32 patients who received narrowband ultraviolet B treatment had complete clearance within an average of 8.9 ± 5.3 months, but 7 patients relapsed within 14.9 ± 14.8 months. One patient with solitary MF failed multiple treatment modalities before eventually achieving disease clearance with photodynamic therapy. Hypopigmented MF is the most common MF variant in Asian children. The diagnostic difficulty is in differentiating this from PLC, which may coexist with MF. Long-term prognosis is generally favorable. [ABSTRACT FROM AUTHOR]

Background There are few studies comparing pityriasis lichenoides (PL) in adults and children, with fewer involving Asians. We compared the clinical profile and treatment outcomes of 10 adults and five children diagnosed with PL. Methods We retrospectively reviewed a series of patients diagnosed with PL at our hospital from 2002 to 2008. Results We identified 10 adults and five children (nine adults and four children with pityriasis lichenoides chronica; one adult and one child with pityriasis lichenoides et varioliformis acuta). Mean age of onset of disease was 42.2 years in adults and 12.2 years in children. Median duration of disease before presentation was 5.5 months in adults and 6 months in children. Trunk and limbs were the most common sites of involvement. Itch was present in the majority. Almost all patients did not respond to topical corticosteroids. A good response was seen in three adults and two children treated with oral antibiotics. Three adults and two children responded to treatment with narrow-band ultraviolet B phototherapy. The median time to resolution was 8 months in adults and 21 months in children. Postinflammatory dyspigmentation was seen in six adults and four children. Conclusion PL presents similarly in adults and children; however, it runs a longer course in children. In our series, postinflammatory hyperpigmentation was common, which may be explained by our patients' darker skin phototypes. We routinely prescribe oral antibiotics initially in patients without contraindications. Phototherapy may be useful in patients not responding to antibiotics. [ABSTRACT FROM AUTHOR]

Aim To describe an Asian population with isolated cutaneous Langerhans cell histiocytosis ( LCH), presenting a review on previous studies on this subgroup of patients and comparing our cohort with other studies of cutaneous LCH with systemic involvement. Methods All patients diagnosed as LCH with cutaneous involvement presenting to a tertiary paediatric hospital ( KK Women's & Children's Hospital) between January 2001 and December 2011 were reviewed. Information recorded included clinical presentation, investigation results, treatment and outcome. Results We identified 10 patients with cutaneous manifestations of LCH. Six had isolated cutaneous LCH with no visceral or bony involvement, while four had cutaneous lesions with involvement of other organ systems. Of the patients with isolated skin involvement, three were treated with surgical excision, one with topical corticosteroids and two with multi-agent chemotherapy. The four patients with concomitant systemic involvement were treated with multi-agent chemotherapy. None of our patients with isolated cutaneous LCH progressed to multisystem disease during the follow-up period. Conclusion Cutaneous LCH, with or without other organ involvement at diagnosis has a good overall prognosis. However, long-term follow-up is recommended in view of possible recurrence. [ABSTRACT FROM AUTHOR]

We present the case of a 5-year-old boy who presented with systematized epidermal nevus with evidence of epidermolytic hyperkeratosis on skin biopsy. He was otherwise well, with normal growth and development and no evidence of other organ system involvement. He had experienced only mild improvement with potent topical corticosteroids. He reported more-marked improvement after 2 months of topical calcipotriol/betamethasone dipropionate combination ointment once a day to the lesions. Extensive epidermal nevi can lead to severe cosmetic disfigurement. We recommend a trial of topical calcipotriol/betamethasone dipropionate ointment for patients with extensive epidermal nevus not amenable to surgical treatment. [ABSTRACT FROM AUTHOR]

A 13-year-old boy underwent allogeneic hematopoietic stem cell transplantation ( HSCT) for underlying acute lymphoblastic leukemia and achieved neutrophil engraftment 28 days after HSCT. He developed ichthyosis 6 weeks after HSCT and then keratotic follicular papules, palmoplantar keratoderma, and a seborrheic dermatitis-like eruption 18 weeks after HSCT. From skin biopsies he was diagnosed with eczematoid graft-versus host disease ( GVHD), which showed spongiosis with scattered necrotic keratinocytes. He responded to oral and topical steroids and an increase in cyclosporine dose. Although uncommon, eczematoid GVHD must be considered in children who have undergone HSCT and then develop an atypical eczematous eruption, especially in the absence of a history of atopy. [ABSTRACT FROM AUTHOR]

We present an 11‐year‐old girl who developed neutropenic fever and papulonodular lesions over her limbs and back following chemotherapy for relapsed acute myeloid leukemia. She was found to have cutaneous Trichosporon asahii infection, with no evidence of systemic infection, and responded to treatment with voriconazole. T. asahii infection is rare but often fatal and usually causes disseminated infections in immunocompromised patients. Cutaneous infection is relatively uncommon and rarely occurs in the pediatric population. [ABSTRACT FROM AUTHOR]

A 9-year-old boy presented with 2 weeks of fever; oligoarthritis involving the right elbow, left knee and elbow; and bilateral conjunctivitis. Reiter's syndrome, also known as reactive arthritis, is characterized by the classical triad of conjunctivitis, arthritis, and urethritis. Our patient was diagnosed with Reiter's syndrome in view of the combination of clinical features of circinate balanitis, arthritis, conjunctivitis, and sterile urethritis. [Extracted from the article]

We report a rare presentation of vulvar lymphangiectasia that developed secondary to gastrointestinal tuberculosis in a teenager, and its spontaneous resolution after anti‐tuberculous treatment was completed. [ABSTRACT FROM AUTHOR]

Narrowband ultraviolet B (NBUVB) phototherapy is a well-established treatment modality for psoriasis. We performed a retrospective analysis of children of East Asian descent with psoriasis treated with NBUVB phototherapy at the National Skin Centre, Singapore, over a 5-year period between 2004 and 2008 and found that NBUVB phototherapy is safe and effective for the treatment of psoriasis in children of East Asian descent. [ABSTRACT FROM AUTHOR]