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Incontinentia pigmenti in Infant/Neonate
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Incontinentia pigmenti in Infant/Neonate

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Contributors: Mehdi Rashighi MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

Incontinentia pigmenti (Stage 1) : Linear configuration, Vesicles, Erythematous plaques
Stage 1
Incontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is an X-linked dominant disease mainly of females that is lethal in males, caused by a mutation in the IKBKG gene. The disease is noticed at birth or within the first few weeks of life. The disease typically progresses through 4 stages: vesicular, verrucous, hyperpigmentation, and hypopigmentation / atrophy. Most cases are associated with developmental abnormalities of the skin, hair, nails, teeth, eyes, and central nervous system. Intellectual disability and epilepsy can be associated. Ocular findings, if present, include both retinal and nonretinal abnormalities, usually related to vascular occlusion.

Codes

ICD10CM:
Q82.3 – Incontinentia pigmenti

SNOMEDCT:
367520004 – Incontinentia pigmenti syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:01/15/2018
Last Updated:01/17/2022
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Incontinentia pigmenti in Infant/Neonate
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A medical illustration showing key findings of Incontinentia pigmenti (Stage 1) : Linear configuration, Vesicles, Erythematous plaques
Clinical image of Incontinentia pigmenti - imageId=119964. Click to open in gallery. caption: 'The vesicular stage, showing patterned erythematous plaques and fine vesiculation on the arm.'
The vesicular stage, showing patterned erythematous plaques and fine vesiculation on the arm.

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