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DeCS
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Descriptor English:
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Malignant Atrophic Papulosis
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Descriptor Spanish:
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Papulosis Atrófica Maligna
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Descriptor Portuguese:
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Papulose Atrófica Maligna
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Synonyms English:
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Degos Disease
Kohlmeier-Degos Disease
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Tree Number:
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C14.907.137.520
C14.907.940.530
C17.800.862.457
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Definition English:
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Variously described as a vasculopathy, endovasculitis, or occlusive arteriopathy, this condition occurs in a benign cutaneous form and a lethal multiorgan systemic variant. It is characterized by a narrowing and occlusion of the lumen of small to medium-sized blood vessels, leading to ischemia and infarction in the involved organ systems. The etiology and pathophysiology are unknown. |
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Indexing Annotation English:
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malignant does not refer to neoplasm; do not confuse with the neoplasm ACANTHOMA, DEGOS see ACANTHOMA
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History Note English:
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2008
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Allowable Qualifiers English:
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Record Number:
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52617
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Unique Identifier:
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D054853
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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