Xeroderma Pigmentosum, Malignant Melanoma, and Congenital Ichthyosis

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Abstract
A medicogenetic and pathologic study was made of a family wherein five of seven siblings manifested xeroderma pigmentosum (XDP); four showed congenital ichthyosis in association with XDP, and of these two had the complication of malignant melanoma. One sibling showed only XDP. Another sibling was clinically free of XDP, but showed histologic evidence of this disease and manifested unequivocal evidence of congenital ichthyosis. Only one other example of the association of XDP and congenital ichthyosis was found. This family is of further interest because of the occurrence of malignant melanoma in two of these siblings; review of the world literature showed that malignant melanoma is an important complication of XDP though this point is rarely emphasized.

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