Darier's disease (original) (raw)
داء دارييه (بالإنجليزية: Darier's disease) هو اضطراب جلدي طفحي وراثي في التقرن، يبدأ عادةً في مرحلة الطفولة ومحدد بموروثة جسمية سائدة، وفيه تنشأ الطفرات والحطاطات التقرانية من جريبات الشعر لتشكل رقع ثؤلولية ويابسة.
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| dbo:abstract | داء دارييه (بالإنجليزية: Darier's disease) هو اضطراب جلدي طفحي وراثي في التقرن، يبدأ عادةً في مرحلة الطفولة ومحدد بموروثة جسمية سائدة، وفيه تنشأ الطفرات والحطاطات التقرانية من جريبات الشعر لتشكل رقع ثؤلولية ويابسة. (ar) Beim Morbus Darier handelt es sich um eine erbliche Hautkrankheit. Sie gehört zu den sehr seltenen angeborenen Syndromen mit Keratoderma, einer die Haut betreffenden Verhornungsstörung (Hyperkeratose). Synonyme sind: Dariersche Krankheit; Dyskeratosis follicularis; Dyskeratosis follicularis Darier; Dyskeratosis follicularis Darier-White; Darier-White-Krankheit; Acrokeratosis verruciformis Hopf; Keratosis vegetans; Psorospermosis follicularis vegetans u. a. Die Namensbezeichnungen beziehen sich auf die Erstautoren unabhängiger Erstbeschreibungen aus dem Jahre 1889 durch den französischen Dermatologen Ferdinand-Jean Darier und den US-amerikanischen Arzt James Clarke White (7. Juli 1833 Belfast, Maine, † 5. Januar 1916, Boston). Eventuell handelt es sich um eine Variante der Acrokeratosis verruciformis (Hopf Syndrom). (de) Darier's disease (DAR) is an inherited skin disorder that presents with multiple greasy, crusting, thick brown bumps that merge into patches. It is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier. Darier's is characterized by dark crusty patches on the skin that are mildly greasy and that emit a strong odor. These patches, also known as keratotic papules, keratosis follicularis, or dyskeratosis follicularis, most often appear on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. Mild forms of the disease are the most common, consisting solely of skin rashes that flare up under certain conditions such as high humidity, high stress, or tight-fitting clothes. Short stature, when combined with poorly-formed fingernails that contain vertical striations, is diagnostic even for mild forms of DAR. Symptoms will usually appear in late childhood or early adulthood between the ages of 15 to 30 years and will vary over the course of one's life. (en) La enfermedad de Darier, también conocida como enfermedad de Darier-White, Disqueratosis Folicular o Queratosis Folicular es una enfermedad autosómica dominante descrita por el dermatólogo francés Ferdinand-Jean Darier. Está caracterizada por pápulas hiperqueratósicas en la piel, que en ocasiones pueden infectarse y contener pus. (es) La maladie de Darier, est une , anomalie de l'épiderme, forme d'érythème, principalement folliculaire, prédominant dans les zones séborrhéiques. Elle a été décrite en 1889 par Ferdinand-Jean Darier sous le nom de psorodermose folliculaire végétante. Certains patients s'organisent en association. (fr) La malattia di Darier, o discheratosi follicolare di Lutz-Darier-White, è una genodermatosi a trasmissione autosomica dominante caratterizzata da un'eruzione cutanea costituita da papule ipercheratosiche. (it) Keratosis follicularis of de ziekte van Darier is een zeldzame, genetisch bepaalde, autosomaal dominant erfelijke aandoening van de huid met onvolledige penetrantie, waarbij afwijkingen van de huid optreden in de vorm van een rode uitslag (oneffenheden) geconcentreerd om de haarzakjes. De ziekte is chronisch en komt vaker bij vrouwen voor dan bij mannen. Wereldwijd heeft ongeveer 1 op de 100.000 mensen deze aandoening. (nl) Choroba Dariera (choroba Dariera-White'a, łac. keratosis follicularis, dyskeratosis follicularis, ang. Darier-White disease, Darier's disease, DAR, DD) – genetycznie uwarunkowana choroba skóry, spowodowana zaburzeniem rogowacenia w obrębie mieszków włosowych i poza nimi. Zmiany dotyczą skóry, paznokci i błon śluzowych. W części przypadków obrazowi klinicznemu choroby towarzyszą zaburzenia rozwojowe. (pl) A Doença de Darier é uma disfunção genética descoberta pelo dermatologista francês Ferdinand-Jean Darier. A doença consiste em uma erupção cutânea teimosa que normalmente afeta o tórax,umbigo, pescoço, orelhas e testa, mas pode se desenvolver em outras áreas do corpo (pt) Болезнь Дарье — Уайта (фолликулярный вегетирующий дискератоз) — редкое наследственное аутосомно-доминантное заболевание, которое характеризуется патологическим ороговением (дискератозом) и высыпаниями в виде роговых, преимущественно фолликулярных, папул на себорейных участках кожного покрова. Заболевание впервые было описано в 1889 году независимо друг от друга французским дерматологом Дж. Дарье и американским дерматологом Дж. К. Уайтом. (ru) |
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| dbp:caption | Linear Darier's disease (en) |
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| dbp:name | Darier's disease (en) |
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| dbp:synonyms | Darier disease, Darier–White disease, Dyskeratosis follicularis, and Keratosis follicularis (en) |
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| gold:hypernym | dbr:Disorder |
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| rdfs:comment | داء دارييه (بالإنجليزية: Darier's disease) هو اضطراب جلدي طفحي وراثي في التقرن، يبدأ عادةً في مرحلة الطفولة ومحدد بموروثة جسمية سائدة، وفيه تنشأ الطفرات والحطاطات التقرانية من جريبات الشعر لتشكل رقع ثؤلولية ويابسة. (ar) La enfermedad de Darier, también conocida como enfermedad de Darier-White, Disqueratosis Folicular o Queratosis Folicular es una enfermedad autosómica dominante descrita por el dermatólogo francés Ferdinand-Jean Darier. Está caracterizada por pápulas hiperqueratósicas en la piel, que en ocasiones pueden infectarse y contener pus. (es) La maladie de Darier, est une , anomalie de l'épiderme, forme d'érythème, principalement folliculaire, prédominant dans les zones séborrhéiques. Elle a été décrite en 1889 par Ferdinand-Jean Darier sous le nom de psorodermose folliculaire végétante. Certains patients s'organisent en association. (fr) La malattia di Darier, o discheratosi follicolare di Lutz-Darier-White, è una genodermatosi a trasmissione autosomica dominante caratterizzata da un'eruzione cutanea costituita da papule ipercheratosiche. (it) Keratosis follicularis of de ziekte van Darier is een zeldzame, genetisch bepaalde, autosomaal dominant erfelijke aandoening van de huid met onvolledige penetrantie, waarbij afwijkingen van de huid optreden in de vorm van een rode uitslag (oneffenheden) geconcentreerd om de haarzakjes. De ziekte is chronisch en komt vaker bij vrouwen voor dan bij mannen. Wereldwijd heeft ongeveer 1 op de 100.000 mensen deze aandoening. (nl) Choroba Dariera (choroba Dariera-White'a, łac. keratosis follicularis, dyskeratosis follicularis, ang. Darier-White disease, Darier's disease, DAR, DD) – genetycznie uwarunkowana choroba skóry, spowodowana zaburzeniem rogowacenia w obrębie mieszków włosowych i poza nimi. Zmiany dotyczą skóry, paznokci i błon śluzowych. W części przypadków obrazowi klinicznemu choroby towarzyszą zaburzenia rozwojowe. (pl) A Doença de Darier é uma disfunção genética descoberta pelo dermatologista francês Ferdinand-Jean Darier. A doença consiste em uma erupção cutânea teimosa que normalmente afeta o tórax,umbigo, pescoço, orelhas e testa, mas pode se desenvolver em outras áreas do corpo (pt) Болезнь Дарье — Уайта (фолликулярный вегетирующий дискератоз) — редкое наследственное аутосомно-доминантное заболевание, которое характеризуется патологическим ороговением (дискератозом) и высыпаниями в виде роговых, преимущественно фолликулярных, папул на себорейных участках кожного покрова. Заболевание впервые было описано в 1889 году независимо друг от друга французским дерматологом Дж. Дарье и американским дерматологом Дж. К. Уайтом. (ru) Darier's disease (DAR) is an inherited skin disorder that presents with multiple greasy, crusting, thick brown bumps that merge into patches. It is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier. Darier's is characterized by dark crusty patches on the skin that are mildly greasy and that emit a strong odor. These patches, also known as keratotic papules, keratosis follicularis, or dyskeratosis follicularis, most often appear on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. (en) Beim Morbus Darier handelt es sich um eine erbliche Hautkrankheit. Sie gehört zu den sehr seltenen angeborenen Syndromen mit Keratoderma, einer die Haut betreffenden Verhornungsstörung (Hyperkeratose). Synonyme sind: Dariersche Krankheit; Dyskeratosis follicularis; Dyskeratosis follicularis Darier; Dyskeratosis follicularis Darier-White; Darier-White-Krankheit; Acrokeratosis verruciformis Hopf; Keratosis vegetans; Psorospermosis follicularis vegetans u. a. Eventuell handelt es sich um eine Variante der Acrokeratosis verruciformis (Hopf Syndrom). (de) |
| rdfs:label | داء دارييه (ar) Darier's disease (en) Morbus Darier (de) Enfermedad de Darier (es) Maladie de Darier (fr) Malattia di Darier (it) Keratosis follicularis (nl) Choroba Dariera (pl) Doença de Darier (pt) Болезнь Дарье — Уайта (ru) |
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