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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. Though the consistency of the skin findings have led to the term "hypomelanosis of Ito", it actually refers to a group of disorders with various genetic causes including polyploidies and aneuploidies. Based upon the specifics of the genetic defect, the skin findings can be accompanied by a great range of systemic findings. These include central nervous system, ocular, and musculoskeletal defects. Nonetheless, the vast majority of cases are limited to the skin. As opposed to incontinentia pigmenti, hypomelanosis of Ito affects both genders equally. This disorder was first described by

AttributesValues
rdf:type
rdfs:label
  • سلس الصباغ المنقص التلون (ar)
  • Hypomelanosis Ito (de)
  • Incontinentia pigmenti achromians (en)
  • Ipomelanosi di Ito (it)
  • Hypomélanose de Ito (fr)
  • Hipomelanoza Ito (pl)
  • Hipomelanose (pt)
rdfs:comment
  • L’hypomélanose de Ito (HI), anciennement dénommée incontinentia pigmenti achromians est une maladie neuro-cutanée caractérisée par des lésions cutanées hypopigmentées le long des lignes de Blaschko qui se développent dans les deux premières années de vie, associées à des symptômes neurologiques, squelettiques et ophtalmologiques. (fr)
  • L'ipomelanosi di Ito, chiamata anche incontinenza achromians pigmenti, è una rara malattia neurocutanea multisistemica caratterizzata da lesioni cutanee ipopigmentate lungo le linee di Blaschko che si sviluppano nei primi due anni della vita, associati a sintomi neurologici, scheletrici o oculari. Deve il nome al dermatologo giapponese Minor Ito che la descrisse nel 1951.L'ipomelanosi di Ito è normalmente caratterizzata da un'insolita leucodermia a spirali, strisce, spruzzi o chiazze, unilaterali o bilaterali, associata a un quadro clinico molto variabile. (it)
  • Hipomelanose é uma alteração dermatológica caraterizada pela redução da melanina, o pigmento que dá a cor saudável da pele. É uma forma de hipocromia e hipopigmentação (pouca cor). Não confundir com vitiligo, uma doença autoimune que caracterizada por partes do corpo com (ausência da pigmentação) com bordas irregulares, assimétricas e bem definidas. Nem confundir com Tinea versicolor, que é uma micose superficial inofensiva que impede partes da pele de bronzear. A melanose geralmente é apenas um problema estético: não causa dor, coceira ou outros sintomas. (pt)
  • Hipomelanoza Ito (łac. incontinentia pigmenti achromians) – rzadka choroba z grupy fakomatoz, charakteryzująca się odbarwionymi plamami i pasmami na skórze, których przebieg jest zgodny z tzw. liniami Blaschko. Objawom skórnym towarzyszą zaburzenia rozwoju kośćca, zębów, włosów i mózgowia, około połowa chorych ma padaczkę. Częste jest opóźnienie umysłowe. Chorobę opisał jako pierwszy japoński dermatolog Ito w 1952 roku. (pl)
  • سلس الصباغ المنقص التلون (بالإنجليزية: Incontinentia pigmenti achromians)‏ ويعروف أيضا باسم ("hypomelanosis of Ito") هو حالة جلدية تتميز بأنماط مختلفة من نقص تصبغ ثنائي أو أحادي الجانب تتبع خطوط بلاشكو. على الرغم من أن اتساق نتائج الجلد قد أدى إلى مصطلح "hypomelanosis of Ito"، فإنه يشير في الواقع إلى مجموعة من الاضطرابات ذات الأسباب الوراثية المختلفة بما في ذلك تعدد الصيغ الصبغية واختلال الصيغة الصبغية. (ar)
  • Die Hypomelanosis Ito (Ito-Syndrom, Incontinentia pigmenti achromians Ito) ist eine sehr seltene, fleckenartige, angeborene Abweichung der färbenden Pigmentzellen der Haut, die bei bestimmten Chromosomzahlabweichungen auftritt. Sie betrifft bestimmte – lebenslang festgelegte – Flecken, an denen eine hellere Färbung der Haut durch lokale Störungen der Pigmentbereitstellung entsteht. Zusätzlich können weitere Störungen auftreten wie zum Beispiel geistige Behinderung, fleckiger Haarausfall, Krampfanfälle. Die Haut zeigt streifenförmige Aufhellungen, die gemustert sind und am Körperstamm und den Armen und Beinen auftreten. Die Muster entstehen durch die geordnete Ausbreitung der genetisch abweichenden Pigmentzellen während der frühen Embryogenese. Sie werden auch Blaschko-Linien genannt. (de)
  • 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. Though the consistency of the skin findings have led to the term "hypomelanosis of Ito", it actually refers to a group of disorders with various genetic causes including polyploidies and aneuploidies. Based upon the specifics of the genetic defect, the skin findings can be accompanied by a great range of systemic findings. These include central nervous system, ocular, and musculoskeletal defects. Nonetheless, the vast majority of cases are limited to the skin. As opposed to incontinentia pigmenti, hypomelanosis of Ito affects both genders equally. This disorder was first described by (en)
differentFrom
name
  • Incontinentia pigmenti achromians (en)
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MeshID
  • D010859 (en)
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synonyms
  • Hypomelanosis of Ito, Linear nevoid hypopigmentation (en)
has abstract
  • سلس الصباغ المنقص التلون (بالإنجليزية: Incontinentia pigmenti achromians)‏ ويعروف أيضا باسم ("hypomelanosis of Ito") هو حالة جلدية تتميز بأنماط مختلفة من نقص تصبغ ثنائي أو أحادي الجانب تتبع خطوط بلاشكو. على الرغم من أن اتساق نتائج الجلد قد أدى إلى مصطلح "hypomelanosis of Ito"، فإنه يشير في الواقع إلى مجموعة من الاضطرابات ذات الأسباب الوراثية المختلفة بما في ذلك تعدد الصيغ الصبغية واختلال الصيغة الصبغية. بناءً على تفاصيل العيب الوراثي، يمكن أن تصاحب نتائج الجلد مجموعة كبيرة من النتائج الجهازية. وتشمل هذه الجهاز العصبي المركزي، والعيوب العينية والعضلية الهيكلية. ومع ذلك، فإن الغالبية العظمى من الحالات تقتصر على الجلد. على عكس الإصابة بسلس الصباغ، يصيب سلس الصباغ المنقص التلون كلا الجنسين بالتساوي. (ar)
  • Die Hypomelanosis Ito (Ito-Syndrom, Incontinentia pigmenti achromians Ito) ist eine sehr seltene, fleckenartige, angeborene Abweichung der färbenden Pigmentzellen der Haut, die bei bestimmten Chromosomzahlabweichungen auftritt. Sie betrifft bestimmte – lebenslang festgelegte – Flecken, an denen eine hellere Färbung der Haut durch lokale Störungen der Pigmentbereitstellung entsteht. Zusätzlich können weitere Störungen auftreten wie zum Beispiel geistige Behinderung, fleckiger Haarausfall, Krampfanfälle. Die Haut zeigt streifenförmige Aufhellungen, die gemustert sind und am Körperstamm und den Armen und Beinen auftreten. Die Muster entstehen durch die geordnete Ausbreitung der genetisch abweichenden Pigmentzellen während der frühen Embryogenese. Sie werden auch Blaschko-Linien genannt. Die Häufigkeit der Erkrankung wird zwischen 1 zu 8.000 und 1 zu 10.000 angegeben. Sie wurde nach dem Erstbeschreiber (1952) benannt. (de)
  • 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. Though the consistency of the skin findings have led to the term "hypomelanosis of Ito", it actually refers to a group of disorders with various genetic causes including polyploidies and aneuploidies. Based upon the specifics of the genetic defect, the skin findings can be accompanied by a great range of systemic findings. These include central nervous system, ocular, and musculoskeletal defects. Nonetheless, the vast majority of cases are limited to the skin. As opposed to incontinentia pigmenti, hypomelanosis of Ito affects both genders equally. This disorder was first described by Japanese dermatologist Minor Ito in 1952. (en)
  • L’hypomélanose de Ito (HI), anciennement dénommée incontinentia pigmenti achromians est une maladie neuro-cutanée caractérisée par des lésions cutanées hypopigmentées le long des lignes de Blaschko qui se développent dans les deux premières années de vie, associées à des symptômes neurologiques, squelettiques et ophtalmologiques. (fr)
  • L'ipomelanosi di Ito, chiamata anche incontinenza achromians pigmenti, è una rara malattia neurocutanea multisistemica caratterizzata da lesioni cutanee ipopigmentate lungo le linee di Blaschko che si sviluppano nei primi due anni della vita, associati a sintomi neurologici, scheletrici o oculari. Deve il nome al dermatologo giapponese Minor Ito che la descrisse nel 1951.L'ipomelanosi di Ito è normalmente caratterizzata da un'insolita leucodermia a spirali, strisce, spruzzi o chiazze, unilaterali o bilaterali, associata a un quadro clinico molto variabile. (it)
  • Hipomelanose é uma alteração dermatológica caraterizada pela redução da melanina, o pigmento que dá a cor saudável da pele. É uma forma de hipocromia e hipopigmentação (pouca cor). Não confundir com vitiligo, uma doença autoimune que caracterizada por partes do corpo com (ausência da pigmentação) com bordas irregulares, assimétricas e bem definidas. Nem confundir com Tinea versicolor, que é uma micose superficial inofensiva que impede partes da pele de bronzear. A melanose geralmente é apenas um problema estético: não causa dor, coceira ou outros sintomas. (pt)
  • Hipomelanoza Ito (łac. incontinentia pigmenti achromians) – rzadka choroba z grupy fakomatoz, charakteryzująca się odbarwionymi plamami i pasmami na skórze, których przebieg jest zgodny z tzw. liniami Blaschko. Objawom skórnym towarzyszą zaburzenia rozwoju kośćca, zębów, włosów i mózgowia, około połowa chorych ma padaczkę. Częste jest opóźnienie umysłowe. Chorobę opisał jako pierwszy japoński dermatolog Ito w 1952 roku. (pl)
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MeSH ID
  • D010859
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DiseasesDB
  • 31154
MedlinePlus
  • 001461
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