Incontinentia pigmenti

This code represents Incontinentia Pigmenti (Bloch-Sulzberger syndrome), a rare, X-linked dominant neurocutaneous disorder primarily affecting females. It is characterized by distinctive skin lesions that evolve through four stages: vesicular, verrucous, hyperpigmented, and atrophic. The condition can also involve the central nervous system, eyes, and teeth.

Use this code when documentation confirms a diagnosis of Incontinentia Pigmenti based on characteristic clinical findings, genetic testing, or biopsy results. This diagnosis is typically made by a dermatologist or geneticist. Supporting documentation should detail the stages of skin involvement and any associated systemic manifestations.

• Ensure documentation clearly distinguishes Incontinentia Pigmenti from other pigmentary disorders.
• Code any associated neurological, ocular, or dental manifestations separately using appropriate codes.
• Genetic testing results confirming the IKBKG gene mutation can strengthen documentation for this diagnosis.