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scientific articles

Ehlers-Danlos Syndrome (Forme Fruste)

key information

source: Proceedings of the Royal Society of Medicine

year: 1937

authors: Davies JH


This boy aged 8 years, otherwise healthy, was first seen in February 1936, complaining of bleeding from vascular tumours on the knees. These had been appearing since he began to walk. An ” angioma ” was removed from the left knee by a surgeon in May 1934. A section from this showed large blood-filled spaces, new-vessel formation and much young fibrous tissue. Studied retrospectively it was partly responsible for a diagnosis of Kaposi’s idiopathic sarcoma. Contemporary sections had the structure of a keloid, containing cyst-like cavities filled with blood. or empty and lined with irregularly proliferating endothelium.

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