---
title: Xeroderma pigmentosum (XP)
description: "Xeroderma pigmentosum is a rare inherited defect in DNA repair that causes extreme sensitivity to UV light. Skin and ocular surfaces are particularly vulnerable to damage and cancer. ## Ocular manifestations UV-induced changes accumulate from early childhood. - Chronic conjunctivitis,…"
url: "https://bluefinvision.com/knowledge-base/topics/conditions-diseases/xeroderma-pigmentosum-xp/"
updated: "2025-12-16"
category: "Conditions & Diseases"
---

# Xeroderma pigmentosum (XP)

Xeroderma pigmentosum is a rare inherited defect in DNA repair that causes extreme sensitivity to UV light. Skin and ocular surfaces are particularly vulnerable to damage and cancer.

## Ocular manifestations

UV-induced changes accumulate from early childhood.

- Chronic conjunctivitis, corneal haze, and lid skin atrophy
- Early onset pterygia, pingueculae, and exposure keratopathy
- High risk of conjunctival and eyelid malignancies
- Photophobia and reduced vision if corneal scarring is severe

## Management

Strict photoprotection and surveillance are essential.

- UV-blocking glasses, face shields, and protective clothing from infancy
- Regular dermatology and ophthalmology review for premalignant changes
- Prompt excision of suspicious lesions
- Genetic counselling and family education on lifelong sun avoidance