Conservative management of small bowel perforation in Ehlers-Danlos syndrome type IV

Table 1 Classification of Ehlers-Danlos syndrome

New classification, Villefranche (1997)	Former classification Berlin (1988)	MIM number1	Inheritance	Biochemical defects
Classic	Type I	130000	AD	COL5A1
	Type II	130010		COL5A2
Hypermobility	Type III	130020	AD	Unknown
Vascular	Type IV	130050	AD	COL3A1
Kyphoscoliosis	Type VI	225400	AR	Lysyl hydroxylase
Arthrochalasia	VIIA, VIIB	130060	AD	COL1A1, COL1A2
Dermatosporaxis	VIIC	225410	AR	Type I collagen N-peptidase
Others	V	305200	XR
	VIII	130800	AD
	X	225310	AR
	XI	147900	AD

It’s adapted from Beighton et al[6], Wenstrup et al[17] and Steinmann et al[18].

¹The MIM number is a numerical assignment for inherited diseases, genes and functional segments of DNA. AD: Autosomal dominant; AR: Autosomal recessive; XR: X-linked recessive.