Xeroderma pigmentosum

This code represents xeroderma pigmentosum, a rare, inherited genetic disorder characterized by extreme sensitivity to ultraviolet (UV) light. Individuals with this condition have a severely impaired ability to repair DNA damage caused by UV radiation, leading to a significantly increased risk of skin cancer and other dermatological and neurological abnormalities.

Use this code for patients diagnosed with xeroderma pigmentosum, confirmed by genetic testing or characteristic clinical findings such as severe sunburn after minimal sun exposure, early onset of freckling, and multiple skin cancers at a young age. Documentation should clearly state the diagnosis of xeroderma pigmentosum.

• Do not confuse with other photosensitivity disorders; this code is specific to the genetic defect in DNA repair.
• Code all associated manifestations, such as specific types of skin cancer (e.g., melanoma, squamous cell carcinoma), neurological complications, or ocular involvement, in addition to this primary diagnosis.
• Ensure documentation specifies the confirmed diagnosis of xeroderma pigmentosum, not just general photosensitivity.