title: Morphometric analysis of elastic skin fibres from patients with: cutis laxa, anetoderma, pseudoxanthoma elasticum, and Buschke-Ollendorff and Williams-Beuren syndromes.
abstract: Computed morphometric analysis of elastic skin fibres in patients with cutis laxa, anetoderma, Williams-Beuren syndrome, pseudoxanthoma elasticum (PXE), and Buschke-Ollendorff syndrome, all clinically ascertained, was performed and compared with data obtained from healthy individuals of the same age. The diameters, area fractions (AA%) and volume fractions (VV%) occupied by pre-elastic fibres and dermal elastic fibres were determined. Irrespective of age the diameter of dermal elastic fibres followed a Gaussian distribution for all groups studied. These diameters were taken into consideration for VV% determinations. Compared with data from skin of healthy subjects of similar age range, VV% of pre-elastic fibres was significantly decreased in patients with cutis laxa, anetoderma, Williams-Beuren syndrome, and PXE and undetectable in Buschke-Ollendorff patients. VV% of dermal elastic fibres was four- to fivefold increased in Buschke-Ollendorff syndrome, two- to threefold increased in PXE skin, four- to fivefold decreased in cutis laxa and anetoderma skin and about twofold decreased in Williams-Beuren skin. The diameter of oxytalan fibres was decreased in anetoderma and Williams-Beuren syndrome while oxytalan fibre diameter was unchanged in PXE and cutis laxa. The diameter of dermal elastic fibres was increased in PXE and Buschke-Ollendorff syndrome, but was decreased in anetoderma and Williams-Beuren syndrome and unchanged in cutis laxa. We demonstrated that cutis laxa, anetoderma, Williams-Beuren syndrome, PXE, and Buschke-Ollendorff syndrome could be easily differentiated by morphometric analysis of elastic skin fibres. Thus we propose that morphometric analyses together with skin biopsies are a valuable tool for distinguishing between inherited and/or acquired skin diseases known to display alterations of elastic fibres.
authors.affiliation: Laboratoire de physiopathologie des tissus non-minéralises, Université René Descartes-Paris V, UFR d'Odontolgie, Montrouge, France.
authors.forename: S
authors.initials: S
authors.lastname: Ghomrasseni
authors.forename: M
authors.initials: M
authors.lastname: Dridi
authors.forename: M
authors.initials: M
authors.lastname: Bonnefoix
authors.forename: D
authors.initials: D
authors.lastname: Septier
authors.forename: G
authors.initials: G
authors.lastname: Gogly
authors.forename: B
authors.initials: B
authors.lastname: Pellat
authors.forename: G
authors.initials: G
authors.lastname: Godeau
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D003483:Cutis Laxa / Q000473:pathology; D020405:Dermis / Q000473:pathology*; D003937:Diagnosis, Differential; D004547:Elastic Tissue / Q000473:pathology*; D016326:Extracellular Matrix Proteins / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D010784:Photomicrography; D011561:Pseudoxanthoma Elasticum / Q000473:pathology; D012867:Skin / Q000473:pathology; D018980:Williams Syndrome / Q000473:pathology
chemical_list: D016326:Extracellular Matrix Proteins; C437744:oxytalan