Ehlers-Danlos Type VII-C, or Human Dermatosparaxis

Elastic They are major manifestations of a condition that reached medical attention in the 17th century after being described by van Meek'ren,¹a Dutch surgeon. The disease is now calledEhlers-Danlos syndrome(EDS) after Ehlers²and Danlos,³who, 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 Falls⁴in 1949. Alteration of the structure of connective tissues in these patients was proposed by Jansen⁵in 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 Beighton⁶to propose a classification that was cited and completed by McKusik⁷in 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.⁸Among them is a disease characterized by extreme joint laxity that Haas and Haas⁹had already suggested in 1958 should be considered a clinical entity, one that they calledarthrochalasis multiplex congenita. It is now categorized as EDS type VII.