hypomelanosis of Ito

hy·po·mel·a·no·sis of I·to

[MIM*146150, MIM*308300, MIM*300337]
not a specific entity but rather represents features of many different forms of mosaicism; characterized by unilateral or bilateral hypopigmented macules in whorls, streaks, and patches in a "marble-cake" pattern, variably associated with epidermal nevi, alopecia, and ocular, skeletal, and neural abnormalities.
See also: incontinentia pigmenti.
Farlex Partner Medical Dictionary © Farlex 2012


Minor, Japanese dermatologist, 1884-1982.
hypomelanosis of Ito - inherited hypopigmented macules variably associated with epidermal nevi, alopecia, and ocular, skeletal, and neural abnormalities. Synonym(s): incontinentia pigmenti achromians
Ito nevus - pigmentation of skin innervated by lateral branches of the supraclavicular nerve and the lateral cutaneous nerve of the arm.
Medical Eponyms © Farlex 2012
References in periodicals archive ?
These groups of diseases, termed as Blaschkoid dyspigmentation [8] include Linear and Whorled Nevoid Hypermelanosis (LWNH), incontinentia pigmenti, linear epidermal nevus, hypomelanosis of Ito and Goltz syndrome etc.
Out of the 5 cases of hypomelanosis of Ito all were generalized, 2 had unilateral involvement and the rest 3 had bilateral distribution.
Hypomelanosis of Ito, described in 1952 as incontinentia pigmenti achromians, is a rare neuroectodermal disorder characterized by mainly hypopigmented and in some cases hyperpigmented anomalies following the Blaschko lines associated with neurological, ocular, and musculoskeletal abnormalities.
The distribution and symmetry of the lines allow the differential diagnosis with entities such as hypomelanosis of Ito, incontinentia pigmenti, linear epidermal nevus and lichen striatus.
Such enlargement of the PVS can also be seen in Lowe syndrome [5] and hypomelanosis of Ito. [6] Lacunar infarcts, cystic periventricular leukomalacia, multiple sclerosis, cryptococcosis and neuro-epithelial cysts may also mimic enlarged PVS.
Hypomelanosis of Ito presents as developmental delay plus swirls or patches of hypopigrnentation or depigmentation along the lines of Blaschko, Dr.
Other anomalies included confirmed or probable incontinentia pigmenti (10.6%); confirmed or probable linear and whorled nevoid hypermelanosis (10.6%); hypomelanosis of Ito (7%); combined hyper- and hypopigmentation (7%); and nevus depigmentosus (3.6%).
To the Editor.--We read with interest the article by Xu et al[1] reporting on the occurrence of a primary meningeal rhabdomyosarcoma in a 15-month-old boy with hypomelanosis of Ito (HI).
My 12-year-old daughter was diagnosed with hypomelanosis of Ito (a rare disorder characterized by an unusual lack of skin color affecting many areas of the body and associated with other symptoms, such as mental retardation, seizuresvisual and ocular defects, overgrowth of brain tissue, and/or a small head) at the age of 2 years.
Other dermatoses observed in this study were Lichen striatus, Linear Lichen planus, inflammatory Linear verrucous epidermal nevus, Lichen nitidus, Paederus dermatitis, Hypomelanosis of Ito, Linear and Whorled nevoid hypermelanosis.
The development of lentigines within nevoid hypopigmentation has been reported only rarely.1,2,3 There is a case report of development of lentigines within nevus depigmentosus as a result of NB-UVB therapy which was given as a result of mistaken diagnosis of nevus depigmentosus for segmental vitiligo.4 In some patients with hypomelanosis of Ito or piebaldism, acquired pigmented macules may occur.
Hypomelanosis of Ito ("incontinentia pigmenti achromians").