[Hypermobile syndrome: functional and aesthetic damages. Observations on 50 cases].
BACKGROUND
Type III Ehlers-Danlos syndrome (EDS III) is a collagen defect and manifests as hypermobile joints, which cause dislocations, and fragile skin, which causes scars. There are three clinical-ultrasound (US) stages.


OBJECTIVE
Diagnostic techniques are described that permit assessment of cutaneous and phalangeal impairment, and job fitness of patients with EDS III.


METHODS
The Beighton scoring system (Bss) and the ultrasound test (US) of the skin and phalanxes (with Sonora Logic 400 MD and 7.5-10 MHz probes) were used in the diagnosis of 50 patients. Dislocations and scars were considered as end-point surrogates and were correlated (in a graph) to clinical-US stages and phalangeal mobility, in order to assess job fitness.


RESULTS
Bss and US results agreed with the literature and differentiated the EDS III into 3 clinical stages. Dislocations involved the wrist-hand (52%), shoulder-arm (16%) and foot-ankle (32%) joints. Spearman test for correlation between joint space and scars, dislocations and phalangeal mobility showed high significance (p<0.0001%).


CONCLUSIONS
Dislocations and scars are useful end-point surrogates in the assessment of job fitness. The correlation between Bss and US findings offers a qualitative clinical evaluation (3 clinical stages), while the graph expresses a quantitative evaluation of the biological impairment. The increase in joint mobility produces positive physiological effects (increase in agility) and negative effects (dislocations, scars) that recommend avoiding excessive load on the joints.
Dipartimento di Dermatologia, Seconda Università di Napoli. Iuraderm@Libero.It
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Iurassich
D000328:Adult; D002921:Cicatrix / Q000209:etiology; D004535:Ehlers-Danlos Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D004204:Joint Dislocations / Q000209:etiology; D008297:Male; D008875:Middle Aged