Xeroderma pigmentosum

Early diagnosis of xeroderma pigmentosum is essential to establish rigorous protection from daylight and careful patient management since child. Less than 40% of patients survive beyond age 20 years. Two important causes of mortality are metastatic malignant melanoma and squamous cell carcinoma.¹⁶ However, well informed patients that accomplish avoiding UV exposure strictly, can expect a longer life-span.

The risk of developing skin cancer will not disappear in sun exposed areas but regular check-ups will help diagnosing them early and treating them before spreading.

¹⁶Schaffer, J. V. & Orlow, S. J. Radiation Therapy for High-Risk Squamous Cell Carcinomas in Patients with Xeroderma Pigmentosum: Report of Two Cases and Review of the Literature. Dermatology 223, 97–103 (2011).