Cutaneous angiosarcoma in a patient with xeroderma pigmentosum

• Main Authors: Arora Raman, Sharma Alok, Gupta Ruchika, Vijayaraghavan M
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2008-10-01
• Series: Indian Journal of Pathology and Microbiology
• Subjects: Angiosarcoma; cutaneous; ultraviolet radiation; xeroderma pigmentosum
• Online Access: http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=4;spage=504;epage=506;aulast=Arora

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disorder characterized by photosensitivity, cutaneous pigmentary changes, premature skin ageing and development of various cutaneous and internal malignancies at an early age as a result of a defect in nucleotide excision repair following ultraviolet light exposure. Cutaneous angiosarcomas are aggressive neoplasms that are rarely associated with XP. In this communication, we report the case of a 40-year-old male patient with XP who developed an angiosarcoma of the face and discuss the implications of this association in view of recent developments in this field.