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

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.

DOI: 10.1007/BF01959394

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Cite this paper

@article{Barabino1990AnAP, title={An atypical paediatric case of malignant atrophic papulosis (K{\"{o}hlmeier-Degos disease)}, author={Arrigo Vittorio Barabino and Ferdinando Pesce and Rosanna Gatti and Palamede Colotto and Flavia Nobili and Rosanna Colacino and Anton Giampalmo}, journal={European Journal of Pediatrics}, year={1990}, volume={149}, pages={457-458} }