Type III Ehlers–Danlos syndrome: correlations among clinical signs, ultrasound, and histologic findings in a study of 35 cases

Iurassich, S; Rocco, Daniela De; Anna, A Starshinova

doi:10.1046/j.1365-4362.2001.01197.x

Cited by 11 publications

(3 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Furthermore, we found that both the classical and the hypermobility types of EDS were accompanied by a reduced thickness of the dermis, which was reliably assessed by 20‐MHz ultrasonography. In a recent study, an analysis of skin thickness in patients with the hypermobility type of EDS performed by 10‐MHz ultrasound showed a decrease in the thickness of the dermis and subcutis together 7 . However, because of the low resolution of 10‐MHz ultrasound, the authors could not attribute this reduction to a specific layer.…”

Section: Discussionmentioning

confidence: 94%

Reduced skin thickness: a new minor diagnostic criterion for the classical and hypermobility types of Ehlers-Danlos syndrome

Eisenbeiss¹,

Martínez²,

Hagedorn‐Greiwe

et al. 2003

Br J Dermatol

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 94%

Reduced skin thickness: a new minor diagnostic criterion for the classical and hypermobility types of Ehlers-Danlos syndrome

Eisenbeiss¹,

Martínez²,

Hagedorn‐Greiwe

et al. 2003

Br J Dermatol

View full text Add to dashboard Cite

show abstract

“…There was a difference in joint width for left elbows, but not for right elbows. A number of studies have reported differences between dominant and non-dominant arms 6,18,25,26 . However, the populations of these studies were comprised of overhead throwing athletes whose dominant and non-dominant elbows are placed under significantly different amounts of repetitive stress.…”

Section: Discussionmentioning

confidence: 99%

Relationship between ulnar collateral ligament thickness and joint hypermobility in young adults

Reuter¹,

Duijn²,

Fichthorn³

et al. 2021

J Orthopedics & Orthopedic Surg

View full text Add to dashboard Cite

Joint hypermobility is largely understood as a dysfunction of collagen fibers within connective tissues, allowing for a range of motion markedly increased over validated normal values. The ulnar collateral ligament (UCL) of the elbow provides stability to the elbow during valgus stress and, thus, has great significance in overhead throwing sports due to its susceptibility to injury. Our cross-sectional study explored the relationship between the length and width of the anterior bundle of the ulnar collateral ligament of the elbow and joint hypermobility. Two hundred and eighty-four undergraduate students completed a Beighton score assessment. Ultrasound images of the participants’ UCL were obtained in both arms at rest and under gravity induced valgus force. Sixty-one participants reported hypermobility in at least one elbow joint, and hypermobility in one elbow joint was correlated with hypermobility in the other. There were moderate correlations between UCL thickness and joint gapping between left and right elbow joints. However, there was no significant difference in UCL anterior bundle thickness or medial elbow joint gapping at rest or under valgus stress between those with and without hypermobile elbow joints. The thickness of the anterior bundle of the UCL and humeroulnar joint gap at rest moderately correlates to corresponding thickness and width under valgus stress. There is no significant difference in UCL anterior bundle thickness or joint width in people with elbow hypermobility and those without.

show abstract

“…12 The PXE echostructure was also different from that in other connective tissue diseases studied with HFUS and could be used for differential diagnosis in ambiguous cases. Several publications have stated that dermal thickness in classical and hypermobile forms of Ehlers-Danlos syndrome is reduced, [18][19][20] although dermal echogenicity is homogeneous.…”

Section: Discussionmentioning

confidence: 99%

High-frequency (20-50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions

et al. 2013

View full text Add to dashboard Cite

Summary Background In most patients pseudoxanthoma elasticum (PXE) manifests with yellowish cutaneous papules and dermal elastorrhexis on skin biopsy. However, in a small number of cases there are no skin manifestations on clinical examination. To establish a diagnosis of PXE in such patients with limited manifestations such as angioid streaks and/or premature cardiovascular disease is challenging. It would therefore be valuable to predict the skin areas that would yield a biopsy specimen positive for elastorrhexis with a non-invasive procedure. High frequency ultrasonography (HFUS) should be evaluated in this respect. Objectives Prior to achieve the goal mentioned above we aimed at describing the characteristics of clinically visible PXE skin using HFUS, and to evaluate its relevance for diagnosis. Methods HFUS was performed in a cohort of PXE patients and controls at a referral centre. HFUS images of PXE skin were compared to those of other conditions. Five operators were tasked with the blind scoring of multiple HFUS images of photoprotected or photoexposed skin from patients with PXE and controls. The diagnostic relevance indices (sensitivity, specificity, likelihood ratios, inter-observer agreement) were calculated. Results The HFUS changes considered as diagnostic for PXE were primarily oval homogeneous hypoechogenic areas in the middermis. The size of these areas closely matched the extent of the histological changes. The sensitivity and specificity of the diagnostic items and inter-observer agreement were high, particularly in photoprotected skin. Dermal hypoechogenicity in PXE could be related to high hydration of connective tissue due to the presence of glycosaminoglycans despite elastic fibre mineralization. Conclusions HFUS provides suggestive images of PXE skin lesions. HFUS should be now studied to determine if it is a potentially valuable technique for the non-invasive identification of elastorrhexis in PXE patients in whom skin involvement is clinically minimal or absent.

show abstract

Type III Ehlers–Danlos syndrome: correlations among clinical signs, ultrasound, and histologic findings in a study of 35 cases

Cited by 11 publications

References 12 publications

Reduced skin thickness: a new minor diagnostic criterion for the classical and hypermobility types of Ehlers-Danlos syndrome

Reduced skin thickness: a new minor diagnostic criterion for the classical and hypermobility types of Ehlers-Danlos syndrome

Relationship between ulnar collateral ligament thickness and joint hypermobility in young adults

High-frequency (20-50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions

Contact Info

Product

Resources

About