Zosteriform lentiginous nevus.
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Biomedical subjects
Publications and source records attributed to W B Reed.
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There are multiple genetic disorders with photosensitivity. The first large group consists of the porphyrias about which a great amount of knowledge has accumulated. The second group is not as well-defined for they are the heredodegenerative disorders of which xeroderma pigmentosum is the most important because here the basic defect in DNA repair is known. Some of the aminoacidurias may cause photosensitivity because of pigment dilution or pellagra-like states. The last group consisting of Darier's, polymorphic light eruption and others are poorly understood, and photosensitivity here is not present in all cases.
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Familial focal facial dermal dysplasia is a disorder, autosomal dominant in its inheritance, characterized by areas of puckered skin at the temples with other areas of the face sometimes affected. The large family reported has 26 known affected individuals. The family considers "cancer of the stomach" as an hereditary trait, but their histories suggest some abdominal carcinoma, such as intestinal polyposis.
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Exposure of normal human skin in vivo to ultraviolet irradiation at wavelengths shorter than 320 nanometers stimtulated an unscheduled DNA synthesis in all of the cell layers of the epidermis and in the upper dermnial fibrocytes. The skin of patients with xeroderma pigmentosum did not show this response. correlation of these findings with previous tissue culture studies suggests that the defect in repair of the damaged DNA in xeroderma cells occurs in vivo as well as in vitro.
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