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 journalArticle

5 Citations (Scopus)

Abstract

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
Volume149
Issue number7
DOIs
Publication statusPublished - Apr 1990

Fingerprint

Malignant Atrophic Papulosis
Blood Vessels
Pediatrics
Toes
Brain Ischemia
Amputation
Skin Diseases
Fingers
Ulcer
Ischemia
Arteries
Skin
Therapeutics

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Barabino, A., Pesce, F., Gatti, R., Colotto, P., Nobili, F., Colacino, R., & Giampalmo, A. (1990). An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease). European Journal of Pediatrics, 149(7), 457-458. https://doi.org/10.1007/BF01959394

An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease). / Barabino, A.; Pesce, F.; Gatti, R.; Colotto, P.; Nobili, F.; Colacino, R.; Giampalmo, A.

In: European Journal of Pediatrics, Vol. 149, No. 7, 04.1990, p. 457-458.

Research output: Contribution to journalArticle

Barabino, A, Pesce, F, Gatti, R, Colotto, P, Nobili, F, Colacino, R & Giampalmo, A 1990, 'An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease)', European Journal of Pediatrics, vol. 149, no. 7, pp. 457-458. https://doi.org/10.1007/BF01959394
Barabino, A. ; Pesce, F. ; Gatti, R. ; Colotto, P. ; Nobili, F. ; Colacino, R. ; Giampalmo, A. / An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease). In: European Journal of Pediatrics. 1990 ; Vol. 149, No. 7. pp. 457-458.
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