Abstract
Hypomelanosis of Ito is a neurocutaneous disorder characterized by hypopigmented whorls, streaks and patches distributed along the lines of Blaschko, often associated with neurological and musculoskeletal abnormalities. We herein report two patients belonging to ethnic Kashmiri origin with this disorder.
Highlights
Incontinentia pigmenti achromians (Ito) first described this condition in 1952
The characteristic features are the presence of hypopigmented skin lesions arranged in whorls and streaks following the lines of Blaschko
Some believe that Hypomelanosis of Ito is related to autosomal dominant inheritance, others attribute it to chromosomal instability and mosaicism [2]
Summary
Ito first described this condition in 1952. Hypomelanosis of Ito is the third most neurocutaneous disorder, after neurofibromatosis and tuberous sclerosis [1]. The characteristic features are the presence of hypopigmented skin lesions arranged in whorls and streaks following the lines of Blaschko. Some believe that Hypomelanosis of Ito is related to autosomal dominant inheritance, others attribute it to chromosomal instability and mosaicism [2]. Chromosomal abnormalities translocation and mosaicism have been reported in approximately 50% cases [3]
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