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Incontinentia Pigmenti Achromians

definition

The term hypomelanosis of Ito (incontinentia pigmenti achromians) has been used as a diagnosis for individuals with hypopigmentation or depigmentation distributed along the lines of Blaschko. The number of bands will vary from one to many and the distribution can be bilateral. Lesions may present at birth or appear during infancy or childhood. In many cases, determination that the lighter areas of skin were hypopigmented rather than the darker areas hyperpigmented has been arbitrary. Approximately two-thirds of these patients have had neurological, skeletal, and/or ocular abnormalities. The prognosis is dependent upon the presence or absence of systemic abnormalities and the severity of these associated anomalies. Evidence documenting single-gene transmission is unconvincing and recurrence risks appear to be negligible in most instances. Karyotyping of blood lymphocytes, skin fibroblasts, and/or keratinocytes of 115 individuals reported in the literature revealed frequent mosaicism.

synonyms

Incontinentia Pigmenti Achromians, Hypomelanosis Ito

UMLS

HYPOMELANOSIS OF ITO, Incontinentia Pigmenti Achromians

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