The previous EDS Type V (X-linked) had been described in a single family. It is a rare variant and the molecular basis of which remains unknown.
The previous EDS Type VIII is similar to the Classical Type, except that in addition it presents with periodontal friability. The existence of this syndrome as an autonomous entity remains uncertain.
The EDS Type IX was previously redefined as “Occipital Horn syndrome”, an X-linked recessive condition allelic to Menkes syndrome. This was previously removed from the EDS classification.
The previous EDS Type X has been described in only one family.
The EDS Type XI termed “Familial Joint Hypermobility syndrome” was previously removed from the EDS classification. Its relationship to the EDS is not yet defined, and it may be a mild variant of Hypermobility Type.
Forms of EDS within this unclassified category may present with soft, mildly stretchable skin, shortened bones, chronic diarrhea, joint hypermobility and dislocation, bladder rupture, or poor wound healing. Inheritance patterns within this group include X-linked recessive, autosomal dominant and autosomal recessive. Examples of these syndromes include: Beasley-Cohen Type, Progeroid form – B4GALT7, Friedman-Harrod Type, Tenascin-X deficiency – TNXB and Musculocontractural type – CHST14.