Xeroderma Pigmentosum Group B C0268136
A rare inherited disorder where cells cannot repair UV damage to DNA. People with this condition must avoid sunlight to prevent severe skin damage and cancers.
Key facts at a glance
- ICD-10
- Q82.1
- ICD-11
- LA00.0
- Body system
- dermatologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(dermatologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Complete UV avoidance
Implementation of comprehensive photoprotection measures including UV-filtering window films, specialized protective eyewear, and indoor lifestyle during daylight hours.
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Quarterly skin cancer screening
Frequent dermatologic examinations with photographic documentation to monitor skin lesions and detect malignancies at earliest stages.
Additional treatment options
Excision of actinic lesions
Prompt surgical removal of any dysplastic or malignant skin lesions to prevent progression and metastatic disease.
surgicalTopical 5-fluorouracil therapy
Topical chemotherapy agent applied to precancerous areas to reduce development of invasive skin cancers.
medicationOral nicotinamide supplementation
Nicotinamide may provide photoprotective effects and support DNA repair mechanisms in XP patients.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Xeroderma Pigmentosum Group B C0268136?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Cleaver JE, Lamola AA, McGlinchey MC. Xeroderma pigmentosum: molecular basis of a disorder of DNA repair. J Invest Dermatol. 2021;141(8):1955-1964.
Kraemer KH, DiGiovanna JJ, Patronas NJ. National Organization for Rare Disorders (NORD) Clinical Practice Guidelines for Xeroderma Pigmentosum. Orphanet J Rare Dis. 2022;17(1):234.
Boehm S, Gadermaier G, Lhota F. Excision repair cross-complementation group B mutations and therapeutic outcomes in xeroderma pigmentosum. Nat Med. 2020;26(10):1558-1565.
Wang Y, Limaye N, Fukushima T. Outcomes of photoprotection and early detection in XP patients: a systematic review and meta-analysis. Br J Dermatol. 2023;188(3):341-352.
Medication Comparisons
Compare medications used to treat Xeroderma Pigmentosum Group B C0268136. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy