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Definition

A form of Ehlers-Danlos syndrome, a group of connective tissue disorders characterized by skin hyperextensibility, articular hypermobility, and tissue fragility. EDSDERMS is an autosomal recessive form characterized by extreme skin fragility and easy bruising, large fontanels, blue sclerae, puffy eyelids, micrognathia, umbilical hernia, and short fingers. Joint hypermobility becomes more important with age.

Acronym

EDSDERMS

Synonyms

Dermatosparaxis
EDS7C
EDS VIIC
Ehlers-Danlos syndrome, type VII, autosomal recessive
Ehlers-Danlos syndrome 7C

Keywords

Ehlers-Danlos syndrome

 
Disclaimer

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