title: High-frequency (20-50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions.
abstract: BACKGROUND
  In most patients pseudoxanthoma elasticum (PXE) manifests with yellowish cutaneous papules and dermal elastorrhexis on skin biopsy. In a small number of cases there are no skin manifestations on clinical examination, and establishing a diagnosis of PXE in such patients is challenging. High-frequency ultrasonography (HFUS) may be of use in predicting skin areas that would yield a biopsy specimen positive for elastorrhexis.
  
  
  OBJECTIVE
  To describe characteristics of clinically visible PXE skin using HFUS, and to evaluate its relevance for diagnosis.
  
  
  METHODS
  HFUS was performed in a cohort of patients with PXE and in controls at a referral centre. HFUS images of PXE skin were compared with those of other conditions. Five operators blind-scored multiple HFUS images of photoprotected or photoexposed skin from patients with PXE and controls. The diagnostic indices (sensitivity, specificity, likelihood ratios, interobserver agreement) were calculated.
  
  
  RESULTS
  The HFUS changes considered as diagnostic for PXE were primarily oval homogeneous hypoechogenic areas in the mid-dermis. The size of these areas closely matched the extent of the histological changes. The sensitivity and specificity of the diagnostic items and interobserver 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 now be studied to determine whether it is a potentially valuable technique for the noninvasive identification of elastorrhexis in patients with PXE in whom skin involvement is clinically minimal or absent.
authors.affiliation: Department of Dermatology, Angers Hospital, University of Nantes Angers Le Mans, Angers, France; PXE Consultation Centre, Angers Hospital, University of Nantes Angers Le Mans, Angers, France.
authors.forename: M
authors.initials: M
authors.lastname: Guérin-Moreau
authors.forename: G
authors.initials: G
authors.lastname: Leftheriotis
authors.forename: Y
authors.initials: Y
authors.lastname: Le Corre
authors.forename: M
authors.initials: M
authors.lastname: Etienne
authors.forename: R
authors.initials: R
authors.lastname: Amode
authors.forename: J F
authors.initials: JF
authors.lastname: Hamel
authors.forename: A
authors.initials: A
authors.lastname: Croué
authors.forename: O
authors.initials: O
authors.lastname: Le Saux
authors.forename: L
authors.initials: L
authors.lastname: Machet
authors.forename: L
authors.initials: L
authors.lastname: Martin
mesh_terms: D000293:Adolescent; D000368:Aged; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D008875:Middle Aged; D015588:Observer Variation; D011561:Pseudoxanthoma Elasticum / Q000000981:diagnostic imaging* / Q000473:pathology; D012680:Sensitivity and Specificity; D012867:Skin / Q000000981:diagnostic imaging / Q000473:pathology* / Q000528:radiation effects; D013472:Sunlight / Q000009:adverse effects; D013473:Sunscreening Agents / Q000494:pharmacology; D014463:Ultrasonography; D055815:Young Adult
chemical_list: D013473:Sunscreening Agents
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