Hypomelanosis of Ito in Child
Alerts and Notices
Synopsis
Neurological findings can be observed in patients with hypomelanosis of Ito including treatment-resistant seizures that develop in infancy, psychomotor developmental delays, and some cases of severe neurologic impairment with hemimegalencephaly. Ocular abnormalities are common and include strabismus, aplasia / hypoplasia of the iris, and heterochromia iridis. Other less common associated findings include cleft lip and palate, anodontia, alopecia, scoliosis, and limb length discrepancy.
Codes
ICD10CM:L81.9 – Disorder of pigmentation, unspecified
SNOMEDCT:
218358001 – Hypomelanosis of Ito
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Vitiligo
- Tuberous sclerosis
- Linear and whorled nevoid hypermelanosis
- Incontinentia pigmenti, fourth stage – Presents with vesicles during the newborn period, progressing to verrucous lesions in early infancy and then hyperpigmentation, finally followed by a hypopigmented phase.
- Nevus depigmentosus
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Management Pearls
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Therapy
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References
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Last Reviewed:04/12/2018
Last Updated:01/17/2022
Last Updated:01/17/2022
Hypomelanosis of Ito in Child