Ehlers-Danlos syndrome is a hereditary collagen disorder
characterized by articular hypermobility, dermal
hyperelasticity, and widespread tissue fragility. Diagnosis is
clinical. Treatment is supportive. Inheritance is usually
autosomal dominant, but Ehlers-Danlos syndrome is heterogeneous.
Different gene mutations affect the amount, structure, or
assembly of different collagens. Mutations can exist in the
genes that encode collagens (eg, type I, III, or V) or collagen-
modifying enzymes (eg, lysyl hydroxylase, a collagen cleaving
protease). The 6 major types are classic, hypermobility,
vascular, kyphoscoliosis, arthrochalasis, and dermatosparaxis.
Thereare also several rare or hard-to-classify types. Symptoms
and Signs Symptoms and signs vary widely. Predominant symptoms
include hypermobile joints, abnormal scar formation and wound
healing fragile vessel, and velvety, hyperextensible skin. Skin
can be stretched several centimeters but returns to normal when
released. Wide papyraceous scars often overlie bony