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LD27.00 Incontinentia pigmenti

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Incontinentia pigmenti is an X-linked dominant gene disorder due to abnormalities of the NF-kappa-B (NEMO) gene on chromosome Xq28. It is lethal in male fetuses but the presence of a normal second X chromosome in females results in a mosaicism which is compatible with life. Affected females present in infancy with skin blisters in linear arrays (Blaschko lines) typically on the scalp and limbs. Within the first few months of life these are succeeded by warty changes and hyperpigmentation. These tend to resolve over time, often leaving atrophic streaks. Associated features include abnormal dentition, ocular defects and a variety of neurological complications.

synonyms

• Incontinentia pigmenti
• Bloch-Siemens syndrome
• Bloch-Sulzberger syndrome
• Incontinentia pigmenti of Bloch-Sulzberger
• nevus pigmentosus systematicus

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