Which of the following statements about Xeroderma Pigmentosa is true?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Xeroderma Pigmentosa (XP) is a genetic disorder characterized by a heightened sensitivity to ultraviolet (UV) rays, primarily due to defects in the nucleotide excision repair (NER) pathway. This defect impairs the body's ability to repair DNA damage induced by UV radiation, particularly from sunlight. As a result, individuals with XP are at significantly increased risk for developing various types of skin cancers, such as melanoma, basal cell carcinoma, and squamous cell carcinoma, particularly in sun-exposed areas of their skin.

The increase in UVB-related skin cancers specifically highlights the consequence of the underlying DNA repair deficiency associated with this condition. Individuals with XP must take extensive precautions to avoid sun exposure to mitigate these risks.

In summary, the statement regarding the increased risk for UVB-related skin cancers accurately reflects the pathophysiology of Xeroderma Pigmentosa, making it the correct choice. The other statements do not accurately represent the nature of the disease, such as its genetic basis or impact on collagen production and immune function.

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