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This syndrome was first described by Lyell (1956) in this country and by Lang and Walker (1956) in South Africa and has since been reported with increasing frequency. Beare (1962), in a comprehensive review, collected 35 cases from the published reports and described 10 further cases that he had observed personally. He suggests that it is unlikely that the syndrome could have been seen in the United Kingdom before 1950 because of its dramatic presentation and considerable mortality and considers that it is occurring more often. It is characterized by an increased skin tenderness and erythema affecting the smooth skin. The hairy parts are often not affected. This is followed within 12 hours by a sudden epidermal necrosis giving rise to blister formation and subsequent scaling. The epidermal separation is followed by a rapid healing of the affected areas. Occasionally burn-like scars are left. The condition has been described in both sexes, the youngest being 2 weeks and the oldest 85 years. The present case is of particular interest as the fully-developed lesions were present at birth.
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Sweetnam et al. (1964) studied this question.
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