A case of Chernogubov-Ehlers-Danlos syndrome

DOI: https://doi.org/10.29296/25877305-2019-12-12
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Issue: 
12
Year: 
2019

Yu. Nefedyeva(1), Candidate of Medical Sciences; Professor O. Ziganshin(1), MD; E. Startseva(2); V. Ivanova(1) 1-South Ural State Medical University, Chelyabinsk 2-Chelyabinsk Regional Clinical Dermatovenereology Dispensary

Ehlers-Danlos syndrome is a rare ontogenetic anomaly in the body’s development, which is one of the challenges to modern medicine. Hyperelastic skin (Chernogubov-Ehlers-Danlos syndrome, imperfect desmogenesis) is a hereditary connective tissue disease caused by insufficient collagen synthesis of various etiologies. Its prevalence is between 1:5,000 and 1:560,000. The paper describes a clinical case in a patient with Ehlers-Danlos syndrome, which includes: his medical history, clinical presentations, and treatment regimen. This clinical case is of interest as a rare syndrome with typical objective signs, such as skin hyperextensibility, prolonged wound healing with scarring, and joint hypermobility.
Keywords: 
Ehlers-Danlos syndrome
skin hyperextensibility
joint hypermobility
diagnosis
treatment

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