Dermatologic, Orthopedic, and Cardiovascular Manifestations and Management in a Geriatric Patient With Dermatosparaxis-Type Ehlers-Danlos Syndrome: A Case Report

Abstract

Ehlers-Danlos syndrome (EDS) is a diverse group of hereditary connective tissue disorders resulting from mutations in genes involved in the synthesis and metabolism of collagens. Collagen, a structural protein in the connective tissues, plays an important role in maintaining the integrity and strength of various tissues, including the skin, ligaments, tendons, cartilage, and blood vessels. As such, EDS is characterized by joint hypermobility, skin elasticity, and tissue fragility. This paper discusses the case of an elderly patient with dermatosparaxis-type EDS (dEDS), a rare autosomal recessive subtype caused by mutations in the ADAMTS2 gene, leading to significant skin fragility, among other characteristic manifestations. This case highlights the complexities involved in the management of the diverse dermatological, orthopedic, and cardiovascular manifestations of dEDS and underscores the importance of individualized care plans that address the complexities of dEDS and improve the quality of life of dEDS patients.