. . "D054853" . "931"@en . . . "Degosova nemoc (zn\u00E1m\u00E1 t\u00E9\u017E jako malign\u00ED atrofick\u00E1 papul\u00F3za) je extr\u00E9mn\u011B vz\u00E1cn\u00E1 , kter\u00E1 postihuje sliznice st\u0159edn\u011B velk\u00FDch a mal\u00FDch \u017Eil a tepen, a zp\u016Fsobuje a infarkt tk\u00E1n\u011B. Mezi posti\u017Een\u00E9 krevn\u00ED c\u00E9vy pat\u0159\u00ED ty, kter\u00E9 z\u00E1sobuj\u00ED k\u016F\u017Ei, tr\u00E1vic\u00ED soustavu a centr\u00E1ln\u00ED nervovou soustavu (CNS). To m\u016F\u017Ee m\u00EDt za n\u00E1sledek st\u0159evn\u00ED ischemii ( nebo ), chronick\u00E9 ko\u017En\u00ED l\u00E9ze, o\u010Dn\u00ED l\u00E9ze, c\u00E9vn\u00ED mozkov\u00E9 p\u0159\u00EDhody (tzv. mrtvice), spin\u00E1ln\u00ED l\u00E9ze, , epilepsie, bolesti hlavy nebo . U pacient\u016F s Degosovou nemoc\u00ED byly zaznamen\u00E1ny i nebo ."@cs . . "La maladie de Degos (aussi appel\u00E9e papulose atrophiante maligne) est une vasculopathie extr\u00EAmement rare qui affecte le rev\u00EAtement int\u00E9rieur des petites et moyennes veines, ce qui entra\u00EEne l'occlusion (blocage du vaisseau) et des infarctus du tissu. La maladie prend son nom de Robert Degos qui l\u2019a reconnue comme une entit\u00E9 clinique en 1942, apr\u00E8s qu'elle a \u00E9t\u00E9 d\u00E9crite, en premier, par l'anatomopathologiste autrichien en 1941."@fr . . . "La enfermedad de Degos o papulosis atr\u00F3fica maligna\u200B es una vasculopat\u00EDa extremadamente rara que afecta el recubrimiento interior de los vasos sangu\u00EDneos (tanto venas como arterias) medianos y peque\u00F1os, lo que lleva a la oclusi\u00F3n (bloqueo) y al infarto del tejido.\u200B Los vasos sangu\u00EDneos afectados incluyen a los que suministran a la piel, tracto gastrointestinal, y sistema nervioso central. Esto puede dar lugar a la isquemia del intestino (isquemia mesent\u00E9rica\u200B o colitis isqu\u00E9mica), lesiones de piel cr\u00F3nicas, lesiones oculares, accidente cerebrovascular, lesiones espinales, mononeuritis m\u00FAltiple,\u200B epilepsia, dolores de cabeza o des\u00F3rdenes cognoscitivos. Tambi\u00E9n, se han reportado derrames pleurales o peric\u00E1rdicos.\u200B\u200B No tiene cura conocida, y frecuentemente desemboca en la muerte. El pron\u00F3stico de esta enfermedad puede ser fatal con una supervivencia media de 2 o 3 a\u00F1os,\u200Baunque a veces suele ocurrir una forma benigna que afecta solamente la piel. Existen menos de cincuenta pacientes vivos y certificados alrededor del mundo, y menos de 200\u200B documentados en la literatura m\u00E9dica. Las opciones de tratamiento son escasas, consisten principalmente en antiagregantes plaquetarios o anticoagulantes o inmunosupresores , y el efecto de dichos tratamientos est\u00E1 limitado a los casos reportados. Se ha sugerido que esta no es una patolog\u00EDa aislada, sino el resultado final de varios des\u00F3rdenes vasculares sist\u00E9micos.\u200B\u200B Esta enfermedad debe su nombre a quien la identific\u00F3 como un caso cl\u00EDnico en 1942, luego de que esta fuera descrita por Kohlmeier en 1941.\u200B\u200B"@es . . . . . . . "Choroba Degosa (choroba Degosa-K\u00F6hlmeiera, grudkowato\u015B\u0107 z\u0142o\u015Bliwa zanikowa, ang. Degos\u2019 disease, K\u00F6hlmeier-Degos\u2019 syndrome, malignant atrophic papulosis) \u2013 rzadka uk\u0142adowa zapalna choroba naczy\u0144 o z\u0142o\u015Bliwym przebiegu i nieznanej etiologii. W przebiegu tej choroby uszkodzeniu ulegaj\u0105 naczynia \u017Cylne i t\u0119tnicze ma\u0142ego i \u015Bredniego kalibru, co powoduje g\u0142\u00F3wnie zmiany sk\u00F3rne, w przewodzie pokarmowym i o\u015Brodkowym uk\u0142adzie nerwowym. Cz\u0119\u015B\u0107 autor\u00F3w wyr\u00F3\u017Cnia \u0142agodny wariant sk\u00F3rny i \u015Bmiertelny wariant uk\u0142adowy. Choroba Degosa jest traktowana jako odr\u0119bna jednostka nozologiczna lub zesp\u00F3\u0142 objaw\u00F3w wsp\u00F3lny dla przebiegu wielu innych chor\u00F3b, g\u0142\u00F3wnie tocznia. Zmiany sk\u00F3rne maj\u0105 posta\u0107 czerwonych lub r\u00F3\u017Cowych grudek na rumieniowym pod\u0142o\u017Cu. Grudki ust\u0119puj\u0105 z pozostawieniem blizn o bardzo charakterystycznym (patognomonicznym) porcelanowym \u015Brodku. Chorob\u0119 opisali Robert Degos ze wsp\u00F3\u0142pracownikami w 1942 i Walter K\u00F6hlmeier w 1941. Od tej pory w pi\u015Bmiennictwie opisano oko\u0142o 200 przypadk\u00F3w schorzenia. Opisano wariant rodzinny choroby Degosa. Trzy g\u0142\u00F3wne hipotezy dotycz\u0105ce jej patogenezy to etiologia wirusowa, autoimmunologiczna lub zaburzenia krzepni\u0119cia. Choroba ta jest nieuleczalna."@pl . "447.8" . . . "931"^^ . . 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\u0641\u064A \u0633\u0646\u0629 1942 \u0628\u0639\u062F \u0623\u0646 \u0648\u0635\u0641\u0647 \u0643\u0648\u0644\u064A\u0645\u064A\u064A\u0631 \u0633\u0646\u0629 1941."@ar . . . "Degos disease"@en . "447.8"^^ . . . . . . "Choroba Degosa"@pl . . . "602248"^^ . "\u062F\u0627\u0621 \u062F\u064A\u063A\u0648 (\u0628\u0627\u0644\u0625\u0646\u062C\u0644\u064A\u0632\u064A\u0629: Degos disease)\u200F \u0623\u0648 \u0627\u0644\u0645\u0633\u0645\u0649 \u0628\u0627\u0644\u062D\u0637\u0627\u0637 \u0627\u0644\u0636\u0645\u0648\u0631\u064A \u0627\u0644\u062E\u0628\u064A\u062B \u0647\u0648 \u0627\u0639\u062A\u0644\u0627\u0644 \u0648\u0639\u0627\u0626\u064A \u0646\u0627\u062F\u0631 \u064A\u0635\u064A\u0628 \u0627\u0644\u0623\u0648\u0631\u062F\u0629 \u0648\u0627\u0644\u0634\u0631\u0627\u064A\u064A\u0646 \u0648\u064A\u0646\u062A\u062C \u0639\u0646\u0647 \u0627\u0646\u0633\u062F\u0627\u062F \u0627\u0644\u0623\u0648\u0639\u064A\u0629 \u0627\u0644\u062F\u0645\u0648\u064A\u0629 \u0648\u0627\u062D\u062A\u0634\u0627\u0621 \u0627\u0644\u0623\u0646\u0633\u062C\u0629. \u0642\u062F \u064A\u0635\u064A\u0628 \u0647\u0630\u0627 \u0627\u0644\u0627\u0639\u062A\u0644\u0627\u0644 \u0627\u0644\u0623\u0648\u0639\u064A\u0629 \u0627\u0644\u062F\u0645\u0648\u064A\u0629 \u0627\u0644\u0645\u0633\u0624\u0648\u0644\u0629 \u0639\u0646 \u0646\u0642\u0644 \u0627\u0644\u062F\u0645 \u0625\u0644\u0649 \u0643\u0644 \u0645\u0646 \u0627\u0644\u062C\u0644\u062F \u0648\u0627\u0644\u0642\u0646\u0627\u0629 \u0627\u0644\u0647\u0636\u0645\u064A\u0629 \u0648\u0627\u0644\u062C\u0647\u0627\u0632 \u0627\u0644\u0639\u0635\u0628\u064A \u0641\u064A\u0635\u0627\u0628 \u0627\u0644\u0645\u0631\u064A\u0636 \u0628\u0623\u062D\u062F \u0627\u0644\u0623\u0645\u0631\u0627\u0636 \u0627\u0644\u062A\u0627\u0644\u064A\u0629: \u0627\u0644\u0625\u0642\u0641\u0627\u0631 (\u0625\u0642\u0641\u0627\u0631 \u0627\u0644\u0645\u0633\u0627\u0631\u064A\u0642 \u0623\u0648 \u0625\u0642\u0641\u0627\u0631 \u0627\u0644\u0634\u0628\u0643\u064A\u0629) \u0623\u0648 \u0627\u0644\u0622\u0641\u0627\u062A \u0627\u0644\u062C\u0644\u062F\u064A\u0629 \u0627\u0644\u0645\u0632\u0645\u0646\u0629 \u0623\u0648 \u0623\u0645\u0631\u0627\u0636 \u0627\u0644\u0639\u064A\u0646 \u0623\u0648 \u0627\u0644\u0646\u0648\u0628\u0627\u062A \u0627\u0644\u0642\u0644\u0628\u064A\u0629 \u0623\u0648 \u0623\u0645\u0631\u0627\u0636 \u0627\u0644\u0639\u0645\u0648\u062F \u0627\u0644\u0641\u0642\u0631\u064A \u0623\u0648 \u0627\u0644\u062A\u0647\u0627\u0628 \u0627\u0644\u0623\u0639\u0635\u0627\u0628 \u0627\u0644\u0645\u062A\u0639\u062F\u062F\u0629 \u0623\u0648 \u0627\u0644\u0635\u0631\u0639 \u0623\u0648 \u0627\u0644\u0635\u062F\u0627\u0639 \u0623\u0648 \u0627\u0636\u0637\u0631\u0627\u0628\u0627\u062A \u0627\u0644\u0625\u062F\u0631\u0627\u0643. \u0646\u0633\u0628 \u0647\u0630\u0627 \u0627\u0644\u0645\u0631\u0636 \u0625\u0644\u0649 \u0631\u0648\u0628\u0631\u062A \u062F\u064A\u063A\u0648 \u0627\u0644\u0630\u064A \u062A\u0639\u0631\u0641 \u0639\u0644\u064A\u0647 \u0641\u064A \u0633\u0646\u0629 1942 \u0628\u0639\u062F \u0623\u0646 \u0648\u0635\u0641\u0647 \u0643\u0648\u0644\u064A\u0645\u064A\u064A\u0631 \u0633\u0646\u0629 1941."@ar . . . . . . "Das Degos-Syndrom ist eine seltene Erkrankung der Arteriolen. Nur etwa 130 Erkrankungsf\u00E4lle wurden in der Literatur weltweit beschrieben (Stand 2009). M\u00F6glicherweise besteht jedoch eine weitaus gr\u00F6\u00DFere Dunkelziffer durch nicht erkannte Krankheitsf\u00E4lle. Das Degos-Syndrom ist auch unter den Namen \u201EDegos-Delort-Tricot-Syndrom\u201C, \u201EPapulosis atrophicans maligna\u201C, \u201Emaligne atrophische Papulose\u201C, \u201Eatrophische papulosquam\u00F6se Dermatitis\u201C, \u201EMorbus K\u00F6hlmeier-Degos\u201C und \u201Efatales kutan-intestinales Syndrom\u201C bekannt. Synonyme sind: Maligne atrophische Papulose; Degos-Krankheit; Kohlmeier-Degos-Krankheit"@de . "Malattia di Degos"@it . . . "La enfermedad de Degos o papulosis atr\u00F3fica maligna\u200B es una vasculopat\u00EDa extremadamente rara que afecta el recubrimiento interior de los vasos sangu\u00EDneos (tanto venas como arterias) medianos y peque\u00F1os, lo que lleva a la oclusi\u00F3n (bloqueo) y al infarto del tejido.\u200B Se ha sugerido que esta no es una patolog\u00EDa aislada, sino el resultado final de varios des\u00F3rdenes vasculares sist\u00E9micos.\u200B\u200B Esta enfermedad debe su nombre a quien la identific\u00F3 como un caso cl\u00EDnico en 1942, luego de que esta fuera descrita por Kohlmeier en 1941.\u200B\u200B"@es . "Degos disease"@en . . . . . "29425" . . . . . . . . . . . . . . . . "Degos disease, also known as K\u00F6hlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition caused by blockage of arteries and veins. Individuals with this condition will develop papules. Those diagnosed with this disease may also develop complications due to impairment of internal organs. The exact underlying mechanism is still unknown, and an effective treatment is still being developed. There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease. Most individuals develop symptoms between the ages of 20\u201350; however, cases outside of this age range have been reported as well."@en . . . "Papulose atrofiante maligna"@pt . "derm"@en . . "Degos-Syndrom"@de . . . "29425"^^ . "Degos disease, also known as K\u00F6hlmeier-Degos disease or malignant atrophic papulosis, is an extremely rare condition caused by blockage of arteries and veins. Individuals with this condition will develop papules. Those diagnosed with this disease may also develop complications due to impairment of internal organs. The exact underlying mechanism is still unknown, and an effective treatment is still being developed. There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease. Most individuals develop symptoms between the ages of 20\u201350; however, cases outside of this age range have been reported as well."@en . "derm"@en . . . "602248"^^ . . . . "Maladie de Degos"@fr . . "Degosova nemoc"@cs . . . . . "La malattia di Degos (anche conosciuta come malattia di K\u00F6hlmeier-Degos o papulosi maligna atropica) \u00E8 una vasculopatia estremamente rara che interessa la tonaca interna di arterie e vene di piccole e medie dimensioni. Esita in occlusioni dei vasi con infarto dei relativi territori di irrorazione."@it . . "Enfermedad de Degos"@es . . . "\u0643\u062B\u0631\u0629 \u0627\u0644\u062D\u0637\u0627\u0637\u0627\u062A \u0627\u0644\u0636\u0645\u0648\u0631\u064A\u0629 \u0627\u0644\u062E\u0628\u064A\u062B\u0629"@ar . . . . . . . "La malattia di Degos (anche conosciuta come malattia di K\u00F6hlmeier-Degos o papulosi maligna atropica) \u00E8 una vasculopatia estremamente rara che interessa la tonaca interna di arterie e vene di piccole e medie dimensioni. Esita in occlusioni dei vasi con infarto dei relativi territori di irrorazione."@it . . . . "14130"^^ . . . . "Degosova nemoc (zn\u00E1m\u00E1 t\u00E9\u017E jako malign\u00ED atrofick\u00E1 papul\u00F3za) je extr\u00E9mn\u011B vz\u00E1cn\u00E1 , kter\u00E1 postihuje sliznice st\u0159edn\u011B velk\u00FDch a mal\u00FDch \u017Eil a tepen, a zp\u016Fsobuje a infarkt tk\u00E1n\u011B. Mezi posti\u017Een\u00E9 krevn\u00ED c\u00E9vy pat\u0159\u00ED ty, kter\u00E9 z\u00E1sobuj\u00ED k\u016F\u017Ei, tr\u00E1vic\u00ED soustavu a centr\u00E1ln\u00ED nervovou soustavu (CNS). To m\u016F\u017Ee m\u00EDt za n\u00E1sledek st\u0159evn\u00ED ischemii ( nebo ), chronick\u00E9 ko\u017En\u00ED l\u00E9ze, o\u010Dn\u00ED l\u00E9ze, c\u00E9vn\u00ED mozkov\u00E9 p\u0159\u00EDhody (tzv. mrtvice), spin\u00E1ln\u00ED l\u00E9ze, , epilepsie, bolesti hlavy nebo . U pacient\u016F s Degosovou nemoc\u00ED byly zaznamen\u00E1ny i nebo . Tato nemoc m\u016F\u017Ee b\u00FDt fat\u00E1ln\u00ED a medi\u00E1n p\u0159e\u017Eit\u00ED je 2 a\u017E 3 roky. U n\u011Bkter\u00FDch pacient\u016F v\u0161ak pravd\u011Bpodobn\u011B m\u016F\u017Ee nastat benign\u00ED forma, kter\u00E1 postihuje pouze k\u016F\u017Ei. V sou\u010Dasn\u00E9 dob\u011B je na sv\u011Bt\u011B m\u00E9n\u011B ne\u017E pades\u00E1t zn\u00E1m\u00FDch \u017Eij\u00EDc\u00EDch pacient\u016F s Degosovou nemoc\u00ED a m\u00E9n\u011B ne\u017E 200 jich je zaznamen\u00E1no v l\u00E9ka\u0159sk\u00E9 literatu\u0159e. Mo\u017Enosti l\u00E9\u010Dby jsou omezen\u00E9 a skl\u00E1daj\u00ED se zejm\u00E9na z protidesti\u010Dkov\u00FDch l\u00E9k\u016F (antiplatelet drugs), antikoagulanci\u00ED \u010Di imunosupresiv a efekt t\u00E9to l\u00E9\u010Dby je zn\u00E1m pouze na z\u00E1klad\u011B kazuistiky. Bylo navr\u017Eeno, \u017Ee Degosova nemoc nen\u00ED samostatnou nemoc\u00ED, n\u00FDbr\u017E kone\u010Dn\u00FDm v\u00FDsledkem n\u011Bkolika syst\u00E9mov\u00FDch c\u00E9vn\u00EDch onemocn\u011Bn\u00ED. Onemocn\u011Bn\u00ED je pojmenov\u00E1no po , kter\u00FD ji roku 1942 rozpoznal jako samostatnou klinickou entitu pot\u00E9, co nemoc jako prvn\u00ED roku 1941 popsal Kohlmeir."@cs . . . "Degos disease"@en . . . . . ""@en . . . "1122854787"^^ . . . . . . . . . "4112395"^^ . "D054853"@en . "Das Degos-Syndrom ist eine seltene Erkrankung der Arteriolen. Nur etwa 130 Erkrankungsf\u00E4lle wurden in der Literatur weltweit beschrieben (Stand 2009). M\u00F6glicherweise besteht jedoch eine weitaus gr\u00F6\u00DFere Dunkelziffer durch nicht erkannte Krankheitsf\u00E4lle. Das Degos-Syndrom ist auch unter den Namen \u201EDegos-Delort-Tricot-Syndrom\u201C, \u201EPapulosis atrophicans maligna\u201C, \u201Emaligne atrophische Papulose\u201C, \u201Eatrophische papulosquam\u00F6se Dermatitis\u201C, \u201EMorbus K\u00F6hlmeier-Degos\u201C und \u201Efatales kutan-intestinales Syndrom\u201C bekannt. Synonyme sind: Maligne atrophische Papulose; Degos-Krankheit; Kohlmeier-Degos-Krankheit Erstmals beschrieben wurde die Erkrankung 1940 von Walter K\u00F6hlmeier, einem Wiener Pathologen, als Unterform der Thrombangiitis obliterans. Kurz darauf erkannte der Pariser Dermatologe Robert Degos unabh\u00E4ngig von K\u00F6hlmeiers Beschreibungen, dass es sich um eine eigenst\u00E4ndige Erkrankung handelt. Die Erkrankung ist nicht zu verwechseln mit dem Erythrokeratoderma en cocardes Degos, einer seltenen erblichen Hautkrankheit."@de . "Skin lesions in a person with Degos disease"@en . . "Choroba Degosa (choroba Degosa-K\u00F6hlmeiera, grudkowato\u015B\u0107 z\u0142o\u015Bliwa zanikowa, ang. Degos\u2019 disease, K\u00F6hlmeier-Degos\u2019 syndrome, malignant atrophic papulosis) \u2013 rzadka uk\u0142adowa zapalna choroba naczy\u0144 o z\u0142o\u015Bliwym przebiegu i nieznanej etiologii. W przebiegu tej choroby uszkodzeniu ulegaj\u0105 naczynia \u017Cylne i t\u0119tnicze ma\u0142ego i \u015Bredniego kalibru, co powoduje g\u0142\u00F3wnie zmiany sk\u00F3rne, w przewodzie pokarmowym i o\u015Brodkowym uk\u0142adzie nerwowym. Cz\u0119\u015B\u0107 autor\u00F3w wyr\u00F3\u017Cnia \u0142agodny wariant sk\u00F3rny i \u015Bmiertelny wariant uk\u0142adowy. Choroba Degosa jest traktowana jako odr\u0119bna jednostka nozologiczna lub zesp\u00F3\u0142 objaw\u00F3w wsp\u00F3lny dla przebiegu wielu innych chor\u00F3b, g\u0142\u00F3wnie tocznia."@pl . "La maladie de Degos (aussi appel\u00E9e papulose atrophiante maligne) est une vasculopathie extr\u00EAmement rare qui affecte le rev\u00EAtement int\u00E9rieur des petites et moyennes veines, ce qui entra\u00EEne l'occlusion (blocage du vaisseau) et des infarctus du tissu. La maladie prend son nom de Robert Degos qui l\u2019a reconnue comme une entit\u00E9 clinique en 1942, apr\u00E8s qu'elle a \u00E9t\u00E9 d\u00E9crite, en premier, par l'anatomopathologiste autrichien en 1941."@fr . "K\u00F6hlmeier-Degos disease"@en . .