Xeroderma pigmentosum is a rare inherited disorder of DNA repair, placing affected individuals at markedly elevated risk of developing malignant skin tumours. Clinical management is oriented around minimising that risk and acting decisively when tumours arise.
The core of management involves establishing a definite diagnosis as early in life as possible, combined with rigorous sun protection education delivered by specialist staff. When skin malignancies are identified, early surgical intervention is a central component of care.
Definite diagnosis at an age that is as young as possible, sun protection education provided by professional staff, patient care, and early detection and early excision of malignant skin tumors greatly contribute to the improvement of QOL of patients and their families.
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