Possible simple measures for complex wound healing problems in ehlers-danlos syndrome.

Sir:

Although Hippocrates described the features of Ehlers-Danlos syndrome (EDS), it was named after 2 physicians, viz., Edvard Ehlers from Denmark and Henri-Alexandre Danlos from France, who described it at the turn of the 20th century.[1] It is an inherited disorder of collagen biosynthesis and structure in humans. Joint hypermobility, skin extensibility, abnormal scarring, and tissue friability are the hallmark diagnostic features; however, EDS is underrecognized because when the physical signs are not “classic,” the diagnosis may be elusive. The medical and scientific history of EDS can be seen in 3 phases: clinical characterization, biochemical and molecular genetic analysis, and the use of high-throughput genomic analysis to extend the phenotypes. Over the last years, the characterization of several new EDS variants has broadened insights into the molecular pathogenesis of EDS by implicating genetic defects in the biosynthesis of other extracellular matrix molecules, such as proteoglycans and tenascin-X, or genetic defects in molecules involved in intracellular trafficking, secretion, and assembly of extracellular matrix proteins.[2] Various types of EDS have been distinguished on clinical and genetic grounds after Villefranche classification, which are not yet incorporated into a coherent classification.

Wound healing is delayed and surgical repair may be difficult because of friable tissues. There is no cure, and treatment is supportive, including close monitoring of the digestive, excretory, and particularly the cardiovascular systems. Occupational and physical therapy, bracing, and corrective surgery may help with the frequent injuries.

On searching the literature, we found that few reported encouraging results,[3-5] and we reviewed these reports and tried to find out the reasons for the better result. We would like to share possible simple solutions to the problem of wound healing in patients with EDS (Table 1).

Table 1.

Major Clinical Problems Related to Wound Healing in Patients with EDS and Possible Solutions

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article. The Article Processing Charge was paid for by the authors.