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Clinical examination Full skin examination assess for hyperextensibility of the skin and abnormal scarring The Beighton scale assess for hypermobility of a joint, in which a score of 5 or more indicates generalised joint hypermobility Investigations Skin biopsy for histology, fibroblast culture, and electron microscopy When suspecting kyphoscoliotic EDS, a urine sample is taken to measure the ratio of lysyl-pyridinoline to hydroxylysyl-pyridinoline cross-links Molecular genetic testing confirms the diagnosis, except in the case of hypermobile EDS, which has an unknown genetic basis and so is a clinical diagnosis which must meet all the clinical criteria

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The DIF test is usually negative, although the presence of deposits of complement component C3 in the perilesional skin has been described in some cases.8,9 The pathogenesis of this adverse reaction remains unclear