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Figure 1. Severely affected XP-C siblings from Turkey in family A. (A) The affected proband, XP100TMA, a 20-year-old male and (B) his 16-year-old sister, XP101TMA, began developing skin lesions at 3 years of age. They had cutaneous atrophy, telangiectasia, actinic keratoses and multiple skin cancers including squamous cell carcinomas, basal cell carcinomas and melanomas. (C) Pedigree of family of XP100TMA. Five generations of this consanguineous family are shown. The proband, XP100TMA (solid square), and his affected sister, XP101TMA (solid circle), have an unaffected brother and two sisters (open symbols). The mother (XPH102TMA) is an obligate heterozygote (half solid symbol).





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