Pleomorphic Dermal Sarcoma and 4 Different Types of Skin Cancer in a Boy With Xeroderma Pigmentosum.

Follow-up of patient series suggested that up to 75% of these patients may develop skin cancers. The differential diagnosis includes atypical fibroxanthoma, spindle-cell SCC, leiomyosarcoma, desmoplastic malignant melanoma, and angiosarcoma. Xeroderma pigmentosum (XP) has been established as an autosomal recessive disorder which is caused by the defect in the repair of DNA due to the ultraviolet (UV) light. [Extracted from the article]