Infantile vitiligo and alopecia in immunodysregulation polyendocrinopathy enteropathy X-linked syndrome.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is characterized by failure to thrive, severe chronic diarrhea, neonatal type 1 diabetes or thyroiditis, and eczematous dermatitis. We report a patient with infantile onset IPEX syndrome who developed vitiligo, alopecia, and chronic diarrhea. Awaiting stem cell transplant, he had multiple episodes of sepsis and succumbed at the age of 10 months. The constellation of symptoms is important to prompt clinicians to suspect this rare syndrome as early hematopoietic stem cell transplantation is the only cure for IPEX patients.

Poor sleep quality in children with atopic dermatitis and its effects on behavior: A multicenter cross-sectional study from a low-middle-income country.

BACKGROUND: Sleep disturbance in children with atopic dermatitis (AD) frequently goes unnoticed and can be associated with behavioral challenges. The aims of this study were to determine (a) the prevalence and factors associated with sleep disturbance and behavioral problems and (b) the correlation between sleep disturbance and behavioral problems in children with AD. METHODS: This cross-sectional study involved children aged 4-12 years old with moderate to severe AD. Age and sex-matched healthy children were recruited as the comparison group. The Children's Sleep Habits Questionnaire (CSHQ) and the Strengths and Difficulties Questionnaire (SDQ) were used to assess sleep disturbance and behavioral problems, respectively. Higher scores in both questionnaires signify more disturbance. RESULTS: Seventy patients and 141 controls were recruited. Median (interquartile range) age of patients was 5 (4,8) years. Patients had later sleep time (p < 0.001), longer night awakening (p < 0.001), and shorter sleep duration (p < 0.001) compared to controls. CSHQ total scores and all its domains, except for sleep-disordered breathing were significantly higher in patients compared to controls. Patients also had significantly higher SDQ total difficulties scores in all domains, except for peer problems. Severity of AD was significantly associated with high CSHQ and SDQ scores. There was a moderate positive correlation between the total CSHQ score and total SDQ score in patients (r = 0.532). CONCLUSIONS: Children with moderate-to-severe AD had significantly more sleep disturbance and more behavioral problems than their healthy peers. Sleep disturbance had a positive correlation with behavioral problems. Severity of AD was associated with sleep disturbance and behavioral problems.

An ulcerated giant segmental hemangioma resulting in contracture of the left elbow.

The majority of infantile hemangiomas are benign and will resolve on their own. We report a 4-month-old infant with an ulcerated giant segmental infantile hemangioma involving the left upper limb who developed a contracture of the left elbow despite treatment with oral propranolol, proper wound care, and regular intense physiotherapy. To our knowledge, contracture resulting from an infantile hemangioma has not been reported previously.

Cost-effectiveness study of pediatric atopic dermatitis in Asia: atopiclair vs. regular emollient (AD-ATOP).

BACKGROUND: Atopic dermatitis (AD) is a highly prevalent, chronic relapsing condition in childhood with significant financial burden and impact on the quality of life of patients and caregivers. Proactive maintenance treatment with moisturizing agents is the mainstay AD therapy. OBJECTIVES: The aim of this study was to assess the cost-effectiveness of a non-steroidal barrier cream (Atopiclair), compared to regular emollient in pediatric patients with mild-to-moderate AD. METHODS: A Markov decision model was developed to evaluate the cost-effectiveness of Atopiclair versus regular emollient in 12 Asia-Pacific countries, grouped by income categories based on gross domestic product (GDP) per capita. Data was obtained from structured literature review, expert opinion, fee schedules, and findings from a 2012 survey of 12 Asia-Pacific countries. Analysis was performed a societal perspective. RESULTS: In the base case analysis, Atopiclair was cost-effective against regular emollient, with USD786, USD499, and USD289 in cost savings per year for high, middle, and low-income countries, respectively. Sensitivity analyses showed that Atopiclair remained cost-effective versus regular emollient. CONCLUSIONS: Modelling analysis showed that Atopiclair is a cost-effective treatment compared to regular emollient for mild-to-moderate pediatric AD in the countries included in the study.

Lichen Striatus: An Updated Review.

BACKGROUND: Lichen striatus is a benign dermatosis that affects mainly children. This condition mimics many other dermatoses. OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations of lichen striatus to avoid misdiagnosis, unnecessary investigations, unnecessary referrals, and mismanagement of lichen striatus. METHODS: A search was conducted in June 2023 in PubMed Clinical Queries using the key term "Lichen striatus". The search strategy included all observational studies, clinical trials, and reviews published within the past ten years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of this article. RESULTS: Lichen striatus is a benign self-limited T-cell mediated dermatosis characterized by a linear inflammatory papular eruption seen primarily in children. The onset is usually sudden with minimal or absent symptomatology. The eruption in typical lichen striatus consists of discrete, skin- colored, pink, erythematous, or violaceous, flat-topped, slightly elevated, smooth or scaly papules that coalesce to form a dull red, potentially scaly, interrupted or continuous band over days to weeks. Although any part of the body may be involved, the extremities are the sites of predilection. Typically, the rash is solitary, unilateral, and follows Blaschko lines. In dark-skinned individuals, the skin lesions may be hypopigmented at onset. Nails may be affected alone or, more commonly, along with the skin lesions of lichen striatus. The differential diagnoses of lichen striatus are many and the salient features of other conditions are highlighted in the text. CONCLUSION: Lichen striatus is a self-limited condition that often resolves within one year without residual scarring but may have transient post-inflammatory hypopigmentation or hyperpigmentation. As such, treatment may not be necessary. For patients who desire treatment for cosmesis or for the symptomatic treatment of pruritus, a low- to mid-potency topical corticosteroid or a topical immunomodulator can be used. A fading cream can be used for post-inflammatory hyperpigmentation.

Pinworm (Enterobius Vermicularis) Infestation: An Updated Review.

BACKGROUND: Pinworm infestation is an important public health problem worldwide, especially among children 5 to 10 years of age in developing countries with temperate climates. The problem is often overlooked because of its mild or asymptomatic clinical manifestations. OBJECTIVE: The purpose of this article was to familiarize pediatricians with the diagnosis and management of pinworm infestation. METHODS: A search was conducted in August 2023 in PubMed Clinical Queries using the key terms "Enterobius vermicularis," OR "enterobiasis," OR "pinworm." The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Enterobiasis is a cosmopolitan parasitosis caused by Enterobius vermicularis. It affects approximately 30% of children worldwide and up to 60% of children in some developing countries. Predisposing factors include poor socioeconomic conditions, inadequate sanitation, poor personal hygiene, and overcrowding. Children aged 5 to 14 years have shown the highest prevalence of enterobiasis.. Egg transmission is mainly by the fecal-oral route. Approximately 30 to 40% of infested patients do not show any clinical symptoms of the disease. For symptomatic patients, the most common presenting symptom is nocturnal pruritus ani. The diagnosis of E. vermicularis infection is best established by the cellophane tape test. The sensitivity of one single test is around 50%; however, the sensitivity increases to approximately 90% with tests performed on three different mornings. If a worm is visualized in the perianal area or the stool, a pathological examination of the worm will yield a definitive diagnosis. As pinworms and eggs are not usually passed in the stool, examination of the stool is not recommended. The drugs of choice for the treatment of pinworm infestation are mebendazole (100 mg), pyrantel pamoate (11 mg/kg, maximum 1 g), and albendazole (400 mg), all of the above-mentioned drugs are given in a single dose and repeated in two weeks. Mebendazole and albendazole are both adulticidal and ovicidal, whereas pyrantel pamoate is only adulticidal. Given their safety and effectiveness, mebendazole and albendazole are currently the best available drugs for the treatment of pinworm infestation. For pregnant women, pyrantel is preferred to mebendazole and albendazole. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections because reinfection is common even when effective medication is given. CONCLUSION: In spite of effective treatment of pinworm infestation, recurrences are common. Recurrences are likely due to repeated cycles of reinfection (particularly, autoinfection) because of the short life span of adult pinworms. Good personal hygiene, such as frequent handwashing, especially after bowel movements and before meals, clipping of fingernails, avoidance of finger-sucking, nail-biting, and scratching in the anogenital area, are important preventive measures. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections.