Rare Diseases Symptoms Automatic Extraction

[Malignant atrophic papulosis].

[malignant atrophic papulosis]

A 53-year-old woman had noticed numerous papules on her trunk and proximal parts of the limbs. They had healed with a central scar surrounded by a reddish wall. Four months later she had to undergo laparotomy for ileus, resulting from a perforation in the region of the middle jejunum: it was excised and sutured over. At laparotomy white plaque-like lesions were noted on the serosa of both small and large intestine. Histological examination of the jejunal exudate and of a skin biopsy both demonstrated malignant atrophic papulosis (Köhlmeier-Dégos' disease). Treatment with ticlopidine, a platelet-aggregation inhibitor (250 mg twice daily), was initiated and she has now been free of extracutaneous symptoms for 6 years.

Diseases presenting "skin biopsy" symptom

  • adrenomyeloneuropathy
  • cadasil
  • cutaneous mastocytosis
  • dentinogenesis imperfecta
  • dracunculiasis
  • epidermolysis bullosa simplex
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • fabry disease
  • harlequin ichthyosis
  • inclusion body myositis
  • junctional epidermolysis bullosa
  • kindler syndrome
  • lamellar ichthyosis
  • malignant atrophic papulosis
  • monosomy 21
  • oligodontia
  • primary effusion lymphoma
  • primary hyperoxaluria type 1
  • pyruvate dehydrogenase deficiency
  • sneddon syndrome
  • waldenström macroglobulinemia
  • x-linked adrenoleukodystrophy

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