Hypomelanosis of Ito: A rare cutaneous syndrome

Abstract

Hypomelanosis of Ito, also known as Incontinentia Pigmenti Achromians of Ito (IPA), is a rare cutaneous syndrome clinically characterized by macular hypopigmented whorls, patches, and streaks either unilateral or bilateral following lines of Blaschko. These skin lesions become apparent at birth or during childhood and normally are present on the trunk, occasionally on the extremities, and rarely on the face. Most patients have multisystem involvement and may show chromosomal mosaicism, the most common being neurological, muscular, skeletal, and ocular. We present a male child of 5-years-old with hypopigmented whorls and patches along the lines of Blaschko present on both sides of the trunk since birth. The child had no other systemic abnormality. The importance of this cutaneous condition and its association with other system involvement has been emphasized in this article.