Pediatric dermatology
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Stiff skin syndrome (SSS) is a disease similar to scleroderma with an unknown etiology. Stone-hard areas of skin are observed from birth or in early childhood. In this article we describe a 15-year-old girl with skin hardening and limitation of movement. We diagnosed the case as SSS, of which we have not encountered a similar report in the Turkish literature.
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Pediatric dermatology · Sep 2002
Review Case ReportsAcrodermatitis enteropathica: case report and review of the literature.
Acrodermatitis enteropathica (AE) is a rare hereditary disorder caused by impaired absorption of zinc from the gastrointestinal tract. It is characterized by acral and periorificial dermatitis, alopecia, and diarrhea. ⋯ The patient was given zinc sulfate 40 mg/day and at the end of 1 month his condition had improved significantly. After reviewing the literature we emphasize the important role of zinc in human metabolism and the difference between AE and acquired zinc deficiencies.
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Pediatric dermatology · Jul 2002
Case ReportsCongenital insensitivity to pain in four related Saudi families.
Congenital insensitivity to pain (hereditary sensory and autonomic neuropathy [HSAN] type V) is a rare disorder of pain perception in which pain sensation is absent from birth, with no other neurologic deficits. We report five Saudi patients (three male and two female) age 10 months to 23 years who lacked pain sensation from birth but have normal appreciation of other sensory modalities. They are from four related families who are descended from one grandfather. The patients had sustained many painless injuries resulting in fractures and disfigurement, but otherwise are completely normal.
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Pediatric dermatology · May 2002
Review Case ReportsNeurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review.
Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy-Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. Association of these entities is very unusual and only 10 previous reports were found in the literature. ⋯ The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.
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Pediatric dermatology · Nov 2001
Dermatosurgery using subcutaneous infusion anesthesia with prilocaine and ropivacaine in children.
Pediatric surgical procedures under local anesthesia have been limited by the pain of injections and, because of low body weight, rapidly reached maximum doses. In subcutaneous infusion anesthesia (SIA) highly diluted local anesthetics are administered by flow- and volume-controlled infusion pumps. This article presents a retrospective review of the use of SIA in children undergoing excision of dermatologic problem lesions. ⋯ No side effects of local anesthesia were observed in these pediatric procedures. The additional use of ropivacaine resulted in prolonged postoperative analgesia. SIA in children is a well-accepted, safe anesthesia that in some cases offers an alternative to general anesthesia.