Table 2. [Xeroderma Pigmentosum: Frequency of Select Features]. - GeneReviews®

Feature	% of Persons w/Feature	Comment
Acute sunburn reaction w/minimal UV exposure	60%
Freckle-like lesions on sun-exposed skin	100%
Xerosis & poikiloderma on sun-exposed skin	100%	Extent of xerosis & poikiloderma is dependent on amount of sun/UV exposure.
Ocular abnormalities	90%	Limited to anterior, UV-exposed portion of eyes & lids
Progressive neurologic abnormalities	25%	Primarily in those w/pathogenic variants in ERCC2, ERCC3, ERCC4, ERCC5, or XPA
Cutaneous neoplasia	65%	Frequency & extent of neoplastic lesions is dependent on amount of sun/UV exposure.
Other neoplasias	10- to 20-fold increase	Internal neoplasms incl brain & CNS tumors, hematologic malignancies, breast cancer, tumors of female reproductive tract, papillary thyroid cancer, kidney cancer, & cancer in smokers have been reported.

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