Pediatric dermatology
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Pediatric dermatology · Sep 2011
Case ReportsCongenital plaque-type glomuvenous malformations associated with fetal pleural effusion and ascites.
Glomuvenous malformations are hereditary vascular anomalies, usually without extracutaneous involvement. We report two cases of extensive thoracic plaque-type glomuvenous malformation in newborns who had previously been diagnosed in utero with pleural effusion and ascites, suggesting a pathogenic link between the two conditions.
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Bier spots are a distinct pattern of vascular mottling. Most reported cases are in young adults, with the youngest case in the literature at age 15 years. We report two children, ages 11 and 12 years, with Bier spots. ⋯ Patient 2 was a 12-year-old girl who presented with similar spots on the dorsal aspect of her left forearm and left hand. In both patients, the spots were visible when extremities were placed in a dependent position and disappeared with elevation of the extremity. Both patients were otherwise healthy.
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Pediatric dermatology · Jul 2011
Frequency and severity of diaper dermatitis with use of traditional Chinese cloth diapers: observations in 3- to 9-month-old children.
Chinese cloth diapers differ from disposable diapers in several respects that are central to our understanding of the etiology of diaper dermatitis (DD), yet there are no published reports on the dermatological correlates of this manner of infant care, which is prevalent among the world's second-largest pediatric population. The objective was to determine the prevalence and severity of DD in exclusive users of Chinese cloth diapers. This observational study was conducted during a single home visit to 694 Chinese children who were exclusive users of cloth diapers in five inland cities of China. ⋯ Diaper dermatitis was rare in the buttocks and genital area of the children (<20%) but was common in the perianal and intertriginous regions (50-70%). TEWL and pH were mildly higher in babies with DD than in those without DD in the genital and buttocks area. Diaper dermatitis is common in children who use traditional Chinese cloth diapers, especially in the perianal and intertriginous regions.
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Pediatric dermatology · Jul 2011
Case ReportsCapillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon.
According to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report a case of a 4-month-old boy that presented a vascular lesion on his right buttock with involvement of abdominal wall muscles, abdominal cavity and drainage to primitive iliac by a tortuous drainage vein. ⋯ No response in platelet counts was obtained and one more surgery was perfomed. On this occasion a histopathologic study revealed vascular malformation areas intermingled with areas of kaposiform hemangioendothelioma. This patient demonstrates the Kasabach-Merritt phenomenon with kaposiform hemangioendothelioma arising within a pre-existing combined vascular malformation.
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Pachyonychia congenita is a rare genodermatosis that can affect the larynx. Laryngeal obstruction is very unusual with only a few cases reported. ⋯ Leukokeratosis of her larynx was treated with CO(2) laser on three occasions, with improvement in her respiratory distress after each treatment. This report is the first case of pachyonychia congenita with laryngeal obstruction in which the gene mutation has been established (a deletional mutation in K6a), confirming that laryngeal obstruction can occur in PC-1.