Xeroderma pigmentoso: relato de caso

Abstract

Introduction: Xeroderma pigmentosum (XP) is a rare autosomal recessive disease, characterized by cutaneous hypersensitivity to ultraviolet radiation. The disease can cause cell mutations that result in changes in the skin, delayed cognitive functions and neuropsychomotor development. It also increases the risk of cancer and interferes with quality of life. Objective: This study aims to report the case of a patient with XP who presented aggressive skin tumor and required radical surgery with reconstruction with a regional musculocutaneous flap. Case Report: MMS, female, 33 years old, with XP, accompanied at the Oncology Service of Sergipe Emergency Hospital since May 2006, was submitted to treatment of tumor recurrence in the face of aggressive squamous cell carcinoma (SCC) with enlargement of the surgical margins and block parotidectomy associated with cervical emptying and reconstruction with platysma musculocutaneous flap. Conclusion: The radical surgical treatment with adequate margins associated with lymphadenectomy and reconstruction with a musculocutaneous flap were effective for the cure of cancer and prevented functional and aesthetic sequelae in this case of Xeroderma pigmentosum.