An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease)

A. Barabino, F. Pesce, R. Gatti, P. Colotto, F. Nobili, R. Colacino, A. Giampalmo

Research output: Contribution to journalArticlepeer-review


A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsed artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected.

Original languageEnglish
Pages (from-to)457-458
Number of pages2
JournalEuropean Journal of Pediatrics
Issue number7
Publication statusPublished - Apr 1990


  • Degos disease
  • Köhlmeier
  • Malignant atrophic papulosis
  • Occlusive angiopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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