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July 1959

Ocular Lesions in Xeroderma Pigmentosum: A Case Report of Carcinoma of the Cornea

Author Affiliations

From the Eye Service of Milwaukee Children's Hospital.

AMA Arch Ophthalmol. 1959;62(1):130-133. doi:10.1001/archopht.1959.04220010134016

Xeroderma pigmentosum, also known as Kaposi's disease or lentigo maligna, was first described in 1870. It is a rare, progressive, precancerous disease of the skin and mucous membranes and frequently displays ocular complications. Mental deficiency has been noted in some patients. It tends to occur in the children of consanguineous marriages, being transmitted as a simple recessive character, and several siblings may have the disease. The pathogenic factor underlying the condition is considered to be a reaction of the surface epithelium to an abnormal organic or chemical disturbance within the body. Sunlight is the precipitating agent of this disease.

The patients usually have normal skins at birth. Xeroderma pigmentosum usually appears before the second year of life as a persistent erythema of the face, neck, and arms after exposure to sunlight but differing from ordinary sunburn in that it takes longer to disappear. This is followed by the gradual appearance

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