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October 1993

Ehlers-Danlos Type VII-C, or Human Dermatosparaxis: The Offspring of a Union Between Basic and Clinical Research

Author Affiliations

From the Laboratory of Experimental Dermatology, University of Liège (Belgium).

Arch Dermatol. 1993;129(10):1316-1319. doi:10.1001/archderm.1993.01680310086015

Elastic They are major manifestations of a condition that reached medical attention in the 17th century after being described by van Meek'ren,1 a Dutch surgeon. The disease is now called Ehlers-Danlos syndrome (EDS) after Ehlers2 and Danlos,3 who, in 1901 and 1908, respectively, described two patients who presented with abnormal rheological properties of skin and joints. The inherited pattern of the disease arose from the study of Johnson and Falls4 in 1949. Alteration of the structure of connective tissues in these patients was proposed by Jansen5 in 1955, at the time of the unveiling of the structure and metabolism of collagen. The numerous clinical reports contributed to the establishment of the heterogeneity of the syndrome, allowing Beighton6 to propose a classification that was cited and completed by McKusik7 in the fourth edition of his treatise entitled "Heritable Disorders of Connective Tissue." Thanks to precise clinical classification, genetic studies, and progress in the determination of the molecular basis of some forms of the disease, nine types of EDS can be defined at the present time.8 Among them is a disease characterized by extreme joint laxity that Haas and Haas9 had already suggested in 1958 should be considered a clinical entity, one that they called arthrochalasis multiplex congenita. It is now categorized as EDS type VII.

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