hypomelanosis of ito

Introduction. Hypomelanosis of Ito. Common symptoms reported by people with hypomelanosis of Ito A 22 year old woman presented with a history of uncontrolled generalised tonic clonic seizures and myoclonic jerks since the age of 2 years. Hypomelanosis of Ito is a clinically well-characterized syndrome in which chromosomal instability may be a component. Hypomelanosis of Ito is a neurocutaneous disorder characterized by skin manifestations in a characteristic pattern associated with musculoskeletal and central nervous system symptoms. Alerts and Notices Synopsis Hypomelanosis of Ito (incontinentia pigmenti achromians) is not a distinct entity, but rather a manifestation of many different states of somatic mosaicism displaying in the skin with "whorls" of hypopigmented skin patches. Other symptoms may include varying degrees of learning disability, seizures, increased body hair, scoliosis, and strabismus. Data from.prior case reports in the world's literature are also reviewed. Citing Literature. More recent, larger studies suggest that the difference may not be as large. Hypomelanosis of Ito (HI) is a relatively common disorder with a frequency of 1 in 8000–10,000 patients in a general pediatric hospital and 1 in 1000 patients in a pediatric neurology service (Küster and Konig 1999). Histochemistry, immunohistochemistry, and electron microscopy were performed on biopsy samples … Hypomelanosis of Ito presents as depigmented whorls, patches and streaks which may be bilateral (Case I) or unilateral (Case 11). The etiology of hypomelanosis of Ito remains mysterious. The skin lesions is believed to be genetically determined. Author information: (1)Pediatric Neurology Service, University Hospital La Paz, Madrid, Spain. Criteria for its presumptive and definitive diagnosis are proposed. Hypomelanosis of Ito. Skin lesions usually develop in early infancy and remain unchanged through childhood and beyond. Related; Information; Close Figure Viewer. Etiology. Pascual-Castroviejo I(1), Roche C, Martinez-Bermejo A, Arcas J, Lopez-Martin V, Tendero A, Esquiroz JL, Pascual-Pascual SI. The incidence of hypomelanosis of Ito is estimated to be 1 in 8,000-10,000 people in the general population. a congenital neurocutaneous syndrome, not present at birth but appearing in early life, characterized by whorled, linear, and splatterlike patterns of hypopigmentation, often associated with other abnormalities such as hair loss and ocular,… We here report another case of hypomelanosis of Ito, including the electron microscopic findings of the hypopigmented areas. 2 Mckusick, 1978 3 considered this an autosomal dominant entity, although in our two cases, there is no consangunity of parents. METHODS: Clinical observations included ultraviolet-light-enhanced visualization (ULEV) method. Hypomelanosis of Ito, also named incontinentia pigmenti achromians in the past, is a rare neurocutaneous syndrome described by Ito in 1952 , and characterized by hypopigmented lesions occurring in streaks and whorls located on the trunk, head, or extremities. Assogba, K, Ferlazzo, E, Striano, P. “Heterogeneous seizure manifestations in hypomelanosis of Ito: report of four new cases and review of the literature”. Hypomelanosis of Ito is a pigmentary mosaicism characterized by a clone of skin cells with decreased ability to produce pigment. Incontinentia pigmenti achromians (also known as "hypomelanosis of Ito") is a cutaneous condition characterized by various patterns of bilateral or unilateral hypopigmentation following the lines of Blaschko. Back; Journal Home; Online First; Current Issue; All Issues; Special Issues; About the journal; Journals. Hypomelanosis of Ito (HI) is characterised by hypochromic unilateral skin lesions, hemi‐hypertrophy, mental retardation (MR) and seizures. Hypomelanosis of Ito represents the third most frequent neurocutaneous disease, after neurofibromatosis type 1 and tuberous sclerosis. March 1992. It is characterized by linear nevoid hypopigmentation along the lines of Blashko located on the limbs and the trunk. The familial occurrence was described as … In earlier reports, hypomelanosis of Ito affected women more often than men by a ratio of 2.5:1. The purpose of the present study was to revisit some aspects of Ito's hypomelanosis. Hypomelanosis of Ito (HI) is a syndrome with hypopigmented whorls of skin along the Blaschko lines. Hypomelanosis of Ito (HI) is a relatively common disorder with a frequency of 1 in 8000–10,000 patients in a general pediatric hospital and 1 in 1000 patients in a pediatric neurology service. Hypomelanosis of Ito, also called incontinentia pigmenti achromians, is a rare birth defect that causes streaked, whirled, or mottled patches of light-colored skin. 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