Rare Diseases Symptoms Automatic Extraction

Malignant atrophic papulosis--Degos' syndrome.

[malignant atrophic papulosis]

A typical case of malignant atrophic papulosis is presented. The patient had had a symptomless, pathognomonic skin rash for 6 months and thereafter also multiple intestinal infarctions which caused perforations, peritonitis, and finally death. All therapeutic attempts, including intestinal resection and acetylsalicylic acid combined with dipyridamole, were in vain. Electron microscopy showed 200-250 A large virus-like cytoplasmic inclusions in the endothelial cells, without degenerative or regenerative changes, in a quite fresh papule. It was not possible to culture viruses on skin fibroblasts, but further studies of these inclusions are needed.

Diseases presenting "skin fibroblasts" symptom

  • child syndrome
  • cowden syndrome
  • cystinuria
  • dentinogenesis imperfecta
  • dystrophic epidermolysis bullosa
  • epidermolysis bullosa simplex
  • gm1 gangliosidosis
  • homocystinuria without methylmalonic aciduria
  • krabbe disease
  • malignant atrophic papulosis
  • monosomy 21
  • neonatal adrenoleukodystrophy
  • pyruvate dehydrogenase deficiency
  • werner syndrome
  • x-linked adrenoleukodystrophy
  • zellweger syndrome

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