Xeroderma pigmentosum, malignant melanoma, and congenital ichthyosis. A family study

Abstract

A medicogenetic and pathologic study was made of a family wherein 5 of 7 siblings manifested xeroderma pigmentosum (XDP); 4 showed congenital ichthyosis in association with XDP, and of these 2 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 1 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 2 of these siblings; review of the world literature showed that malignant melanoma is an important complication of XDP though this point is rarely emphasized.