Hypomelanosis of Ito with gynaecomastia and dental anomaly

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Hypomelanosis of Ito

Hypomelanosis of Ito (HI) is a rare neurocutaneous syndrome with characteristic whirled hypopigmented skin lesions and neurological manifestations. Less consistently, there may be non-neurological manifestations that include ophthalmic, musculoskeletal, craniofacial, cardiac, genitourinary, and gastrointestinal involvements. We report a case of HI, with typical skin lesions in association with ...

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[Hypomelanosis of Ito - case report]

OBJECTIVES: The authors report a case of hypomelanosis of Ito (HI), a rare neurocutaneous syndrome with neurological and chromosomal alterations associated with cutaneous involvement and recurrent pneumonia. CASE REPORT: A male patient, age 1 year and 11 months, was admitted with bilateral bronchopneumonia to the São Vicente de Paulo Hospital. Examination revealed hypochromic maculas on the ski...

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Incontinentia pigmenti and hypomelanosis of Ito.

Incontinentia pigmenti (IP) is a rare X-linked dominant neurocutaneous disorder affecting ectodermal tissue: skin, eyes, central nervous system, hair, nails, and teeth. It is usually lethal for males in utero. The involved gene is NEMO, an essential component of the nuclear factor-kappa B (NF-κB) signaling pathway. Skin lesions are highly diagnostic, occurring in neonates, with a particular dis...

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A Case Study of Hypomelanosis of Ito

Among nevoid causes of hypopigmentations; incontinentia pigmenti achromians (Hypomelanosis of Ito) is a neurocutaneous syndromes characterized by distinctive macular, linear or irregular whorls or swirls of Hypopigmentations. Unrelated to incontinentia pigmenti, the loss of pigment begins spon­taneously during infancy or early childhood and is of Particular importance because of the falt that m...

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Hypomelanosis of Ito with Partial Motor Seizure and

Hypomelanosis of I to [HI] or Incontinentia pigmenti achromians [OMIM no. 146150]. Ito first introduced the syndrome 1951 [1]. It is a rare neurocutaneous syndrome that involves mainly skin and nervous system symptoms in 75 % of cases and may be associated with multiple organ systems involvement including the head and face, eyes (microphthalmia, cataracts, optic atrophy, and retinal detachment)...

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ژورنال

عنوان ژورنال: BMJ Case Reports

سال: 2018

ISSN: 1757-790X

DOI: 10.1136/bcr-2018-225055