Congenital absence of skin = غياب الجلد الخلقي |
Congenital absence of skin Congenital absence of skin is a rare condition which was first reported in
1826. Until 1960 only 213 cases hadbeen reported in the literature. It usually presents in the newborn as localized areas of skin deficiency, most commonly involving the scalp. The aetiology of the condition is unknown, but various theories havebeen postulated. One of the earlier theories was that of the amniotic adhesion theory. Intra-uterine trauma because of pressure was also postulated.There is no doubt that hereditary factors play a role because familial histories have been obtained. In those cases where microscopical studies of the lesions were performed, it was found that there was an absence of sebaceous glands, hair papillae and sweat glands in the affected area. There was also an absence of elastic fibres in the corium of the affected area and to a certain extent in the surrounding areas. The condition was therefore compared with epidermolysis bullosa hereditaria, in which there is also believed to be a deficiency of elastic fibres in the corium. Underlying defects of the skull bone are common and may be large.The mortality in cases with scalp de¬fects is about 20%, the main cause of death being menin¬gitis.The treatment in cases where only the scalp is deficient consists of a Thiersch graft and later, if necessary, rotation of scalp flaps. If there is a large defect of the underlying bone the defect should by pre¬ference be covered with a scalp flap,
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