HYPOMELANOSIS OF ITO
By Gary M. White, MD
Hypomelanosis of Ito (HI) describes linear and whorled areas of skin hypopigmentation along the lines of Blaschko.
- Various names have been used including linear and whorled nevoid hypomelanosis, systematized nevus depigmentosus, cutis tricolor and recently, Blaschkoid dyspigmentation [Pediatric Dermatology 31:4, pages 471–476, July/August 2014].
- This author prefers to reserve the term nevus depigmentosus for smaller, limited areas of hypomelanosis.
- The cause is often chromosomal mosaicism or translocation.
- Many anomalies have been associated including psychomotor or mental retardation, autistic behavior, microcephalus, epileptic seizures, musculoskeletal anomalies, ocular anomalies, dental disorders, coarse facies, dysmorphic ears, hypoacusis, congenital heart disease, choanal atresia, cleft palate, segmental dilatation of the colon, cryptorchidism, inguinal hernia, low height, vesicoureteral reflux and premature pubarche.
- Because a small percentage of patients with incontinentia pigmenti may later develop hypopigmented linear and whorled lesions, the diagnosis of HI should only be made in the absence of preceding inflammation or pigmented macules.
- It has been proposed to use the terms HI with systemic involvement, or HI without systemic involvement.
Streaks and whorls of hypopigmentation appearing within the first year of life are characteristic. If the scalp is involved, streaks of white hair may occur. The true incidence of abnormalities is probably around 30%.
Chromosomal analysis may be done. Otherwise, a good history and physical is probably all that is needed.
No specific treatment for the skin is known. Gradual repigmentation may occur.
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