Short answer · Medically reviewed summary · Last updated: 2026-05-08
Incontinentia Pigmenti is classified under ICD-10 code Q82.3 and ICD-9 code 757.33. These billing and diagnostic codes are used internationally to identify this rare, X-linked dominant genetic disorder characterized by multisystem involvement, including dermatological, dental, ocular, and neurological manifestations. What is the clinical significance of Incontinentia Pigmenti? Incontinentia Pigmenti is a rare genodermatosis typically caused by mutations in the IKBKG (NEMO) gene.
ICD10 code of Incontinentia Pigmenti and ICD9 code
ICD-10 and ICD-9 codes for Incontinentia Pigmenti, with classification details for clinicians, coders and patients.
Incontinentia Pigmenti is classified under ICD-10 code Q82.3 and ICD-9 code 757.33. These billing and diagnostic codes are used internationally to identify this rare, X-linked dominant genetic disorder characterized by multisystem involvement, including dermatological, dental, ocular, and neurological manifestations.
What is the clinical significance of Incontinentia Pigmenti?
Incontinentia Pigmenti is a rare genodermatosis typically caused by mutations in the IKBKG (NEMO) gene. Because it is X-linked dominant, it is predominantly seen in females; it is usually lethal in males in utero, except in cases of Klinefelter syndrome (XXY) or somatic mosaicism. The 158 members of our Incontinentia Pigmenti community at DiseaseMaps.org frequently discuss the four distinct stages of skin lesions, which range from inflammatory vesicles to permanent hyperpigmentation.
How is Incontinentia Pigmenti diagnosed and coded?
While the ICD-10 code Q82.3 and ICD-9 code 757.33 are essential for medical documentation and insurance purposes, a definitive diagnosis of Incontinentia Pigmenti relies on clinical criteria and molecular genetic testing. Physicians look for the characteristic skin patterns alongside secondary findings such as hypodontia (missing teeth), retinal vascular abnormalities, and potential developmental delays.
What are the primary clinical features of Incontinentia Pigmenti?
Management of Incontinentia Pigmenti requires a multidisciplinary approach due to the systemic nature of the condition. Patients often require coordinated care across several specialties:
- Dermatology: Monitoring of skin lesions through the four stages (vesicular, verrucous, hyperpigmented, and atrophic).
- Ophthalmology: Regular retinal screenings to prevent or treat potential retinal detachment or ischemia.
- Dentistry: Management of delayed tooth eruption, conical teeth, or hypodontia.
- Neurology: Evaluation for seizures or cognitive delays that occur in a subset of patients.
Next steps
- Consult with a clinical geneticist to confirm the diagnosis of Incontinentia Pigmenti through IKBKG gene sequencing.
- Schedule baseline evaluations with a pediatric ophthalmologist and a dentist familiar with rare ectodermal dysplasias.
- Connect with the 158 members of the Incontinentia Pigmenti community at DiseaseMaps.org to share experiences and coping strategies.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Incontinentia Pigmenti.
- Orphanet: Incontinentia Pigmenti (ORPHA:464).
- OMIM (Online Mendelian Inheritance in Man): Entry #308300 (Incontinentia Pigmenti).
- National Incontinentia Pigmenti Foundation (NIPF).
