Skip to main content
Erschienen in: American Journal of Clinical Dermatology 5/2006

01.10.2006 | Original Research Article

Collagen Fibril Arabesques in Connective Tissue Disorders

verfasst von: Trinh Hermanns-Lê, Prof. Gérald E. Piérard

Erschienen in: American Journal of Clinical Dermatology | Ausgabe 5/2006

Einloggen, um Zugang zu erhalten

Abstract

Background: In various connective tissue disorders, collagen fibrils and elastic fibers may exhibit some ultrastructural abnormalities.
Objective: This electron microscopy study focused on collagen fibril orientation and unusual shapes in the dermis of Ehlers-Danlos syndrome (EDS) and related entities, including spontaneous cervical artery dissection syndrome and recurrent preterm premature rupture of fetal membranes syndrome.
Results: In addition to some abnormal fibril shapes typically found in these syndromes, other conformations (hook shaped, S shaped, ring shaped) that have not been illustrated previously in the literature were observed.
Conclusion: The abnormal collagen fibril conformations observed in EDS and other related conditions characterized by tissue fragility likely affect both the internal cohesiveness of the bundles and the mechanobiologic properties of the tissue.
Literatur
1.
Zurück zum Zitat Vogel A, Holbrook KA, Steinmann B, et al. Abnormal collagen fibril structure in the gravis form (type I) of the Ehlers-Danlos syndrome. Lab Invest. 1979; 40: 201–6PubMed Vogel A, Holbrook KA, Steinmann B, et al. Abnormal collagen fibril structure in the gravis form (type I) of the Ehlers-Danlos syndrome. Lab Invest. 1979; 40: 201–6PubMed
2.
Zurück zum Zitat Holbrook KA, Byers PH. Structural abnormalities in the dermal collagen and elastic matrix from the skin of patients with inherited connective disorders. J Invest Dermatol. 1982; 79 Suppl. 1: 7S–11SPubMedCrossRef Holbrook KA, Byers PH. Structural abnormalities in the dermal collagen and elastic matrix from the skin of patients with inherited connective disorders. J Invest Dermatol. 1982; 79 Suppl. 1: 7S–11SPubMedCrossRef
3.
Zurück zum Zitat Piérard GE, Piérard-Franchimont C, Lapière CM. Histopathology aid at the diagnosis of the Ehlers-Danlos syndrome gravis and mitis types. Int J Dermatol. 1983; 22: 300–4PubMedCrossRef Piérard GE, Piérard-Franchimont C, Lapière CM. Histopathology aid at the diagnosis of the Ehlers-Danlos syndrome gravis and mitis types. Int J Dermatol. 1983; 22: 300–4PubMedCrossRef
4.
Zurück zum Zitat Piérard GE, Lê T, Piérard-Franchimont C, et al. Morphometric study of cauliflower collagen fibrils in Ehlers-Danlos syndrome type I. Coll Rel Res. 1988; 8: 453–7CrossRef Piérard GE, Lê T, Piérard-Franchimont C, et al. Morphometric study of cauliflower collagen fibrils in Ehlers-Danlos syndrome type I. Coll Rel Res. 1988; 8: 453–7CrossRef
5.
Zurück zum Zitat Nusgens BV, Verellen-Dumoulin C, Hermanns-Lê T, et al. Evidence for a relationship between Ehlers-Danlos type VIIC in human and bovine dermatosparaxis. Nat Genet. 1992; 1: 214–7PubMedCrossRef Nusgens BV, Verellen-Dumoulin C, Hermanns-Lê T, et al. Evidence for a relationship between Ehlers-Danlos type VIIC in human and bovine dermatosparaxis. Nat Genet. 1992; 1: 214–7PubMedCrossRef
6.
Zurück zum Zitat Watson RB, Wallis GA, Holmes DF, et al. Ehlers-Danlos syndrome type II B: incomplete cleavage of the patient’s abnormal type I collagen by N-proteinase results in the formation of rough-bordered collagen fibrils characteristic of the disorder. J Biol Chem. 1992; 267: 25529–34 Watson RB, Wallis GA, Holmes DF, et al. Ehlers-Danlos syndrome type II B: incomplete cleavage of the patient’s abnormal type I collagen by N-proteinase results in the formation of rough-bordered collagen fibrils characteristic of the disorder. J Biol Chem. 1992; 267: 25529–34
7.
Zurück zum Zitat Piérard GE, Hermanns-Lê T, Arrese-Estrada J, et al. Structure of the dermis in type VIIC Ehlers-Danlos syndrome. Am J Dermatopathol. 1993; 15: 127–32PubMedCrossRef Piérard GE, Hermanns-Lê T, Arrese-Estrada J, et al. Structure of the dermis in type VIIC Ehlers-Danlos syndrome. Am J Dermatopathol. 1993; 15: 127–32PubMedCrossRef
8.
Zurück zum Zitat Hausser I, Anton-Lamprecht I. Differential ultrastructural aberration of collagen fibrils in Ehlers-Danlos syndrome type I-IV as a mean of diagnostics and classification. Hum Genet. 1994; 3: 394–407 Hausser I, Anton-Lamprecht I. Differential ultrastructural aberration of collagen fibrils in Ehlers-Danlos syndrome type I-IV as a mean of diagnostics and classification. Hum Genet. 1994; 3: 394–407
9.
Zurück zum Zitat Smith TL, Schwarze U, Goldstein J, et al. Mutations in the COL3A1 gene result in the Ehlers-Danlos syndrome type IV and alterations in the size and distribution of the major collagen fibrils of the dermis. J Invest Dermatol. 1997; 108: 241–7PubMedCrossRef Smith TL, Schwarze U, Goldstein J, et al. Mutations in the COL3A1 gene result in the Ehlers-Danlos syndrome type IV and alterations in the size and distribution of the major collagen fibrils of the dermis. J Invest Dermatol. 1997; 108: 241–7PubMedCrossRef
10.
Zurück zum Zitat Hermanns-Lê T. How I explore... some cutaneous disorders by the ultrastructural examination of the skin. Rev Med Liege. 2000; 55: 954–6PubMed Hermanns-Lê T. How I explore... some cutaneous disorders by the ultrastructural examination of the skin. Rev Med Liege. 2000; 55: 954–6PubMed
11.
Zurück zum Zitat Flagothier C, Goffin V, Hermanns-Lê T, et al. A 4-generation Ehlers-Danlos syndrome with vascular dissections: skin ultrastructure and biomechanical properties. J Med Eng Technol. Epub 2006 Mar 29 Flagothier C, Goffin V, Hermanns-Lê T, et al. A 4-generation Ehlers-Danlos syndrome with vascular dissections: skin ultrastructure and biomechanical properties. J Med Eng Technol. Epub 2006 Mar 29
12.
Zurück zum Zitat Brandt T, Hausser I, Orbek E, et al. Ultrastructural connective tissue abnormalities in patients with spontaneous cervicocerebral artery dissections. Ann Neurol. 1988; 44: 281–5CrossRef Brandt T, Hausser I, Orbek E, et al. Ultrastructural connective tissue abnormalities in patients with spontaneous cervicocerebral artery dissections. Ann Neurol. 1988; 44: 281–5CrossRef
13.
Zurück zum Zitat Brandt T, Orbek E, Weber R, et al. Pathogenesis of cervical artery dissections: association with connective tissue abnormalities. Neurology. 2001; 57: 24–30PubMedCrossRef Brandt T, Orbek E, Weber R, et al. Pathogenesis of cervical artery dissections: association with connective tissue abnormalities. Neurology. 2001; 57: 24–30PubMedCrossRef
14.
Zurück zum Zitat Hermanns-Lê T. How I explore... some vascular disorders using ultrastructural assessment of the skin. Rev Med Liege. 2004; 59: 598–600PubMed Hermanns-Lê T. How I explore... some vascular disorders using ultrastructural assessment of the skin. Rev Med Liege. 2004; 59: 598–600PubMed
15.
Zurück zum Zitat Ulbricht D, Diederich NJ, Hermanns-Lê T, et al. Cervical artery dissection: the single clinical sign of a connective tissue disorder?. Neurology. 2004; 63: 1708–10PubMedCrossRef Ulbricht D, Diederich NJ, Hermanns-Lê T, et al. Cervical artery dissection: the single clinical sign of a connective tissue disorder?. Neurology. 2004; 63: 1708–10PubMedCrossRef
16.
Zurück zum Zitat Hermanns-Lê T, Piérard GE, Quatresooz P. Ehlers-Danlos-like dermal abnormalities in women with recurrent preterm premature rupture of the fetal membranes. Am J Dermatopathol. 2005; 27: 407–10PubMedCrossRef Hermanns-Lê T, Piérard GE, Quatresooz P. Ehlers-Danlos-like dermal abnormalities in women with recurrent preterm premature rupture of the fetal membranes. Am J Dermatopathol. 2005; 27: 407–10PubMedCrossRef
17.
Zurück zum Zitat Piérard GE, Lê T, Hermanns JF, et al. Morphometric study of cauliflower collagen fibrils in dermatosparaxis of the calves. Coll Rel Res. 1986; 6: 481–92CrossRef Piérard GE, Lê T, Hermanns JF, et al. Morphometric study of cauliflower collagen fibrils in dermatosparaxis of the calves. Coll Rel Res. 1986; 6: 481–92CrossRef
18.
Zurück zum Zitat Kobayasi T, Ullman S. Twisted collagen fibrils: significance for diagnosis of Ehlers-Danlos syndrome [abstract]. J Invest Dermatol. 1996; 107: 266 Kobayasi T, Ullman S. Twisted collagen fibrils: significance for diagnosis of Ehlers-Danlos syndrome [abstract]. J Invest Dermatol. 1996; 107: 266
19.
Zurück zum Zitat Kobayasi T, Ullman S. Acrocyanosis and Ehlers-Danlos syndrome. Eur J Dermatol. 1995; 5: 167–8 Kobayasi T, Ullman S. Acrocyanosis and Ehlers-Danlos syndrome. Eur J Dermatol. 1995; 5: 167–8
20.
Zurück zum Zitat Kobayasi T, Ullman S. Twisted collagen fibrils in acrocyanosis. Eur J Dermatol. 1999; 9: 285–8PubMed Kobayasi T, Ullman S. Twisted collagen fibrils in acrocyanosis. Eur J Dermatol. 1999; 9: 285–8PubMed
21.
Zurück zum Zitat De Moraes AM, Cintra ML, de Almeida Prado Sampaio S, et al. The ultrastructural and histophotometric study of elastic and collagen fibers in type II Ehlers-Danlos syndrome and subclinical forms. Ultrastruct Pathol. 2000; 24: 129–34PubMedCrossRef De Moraes AM, Cintra ML, de Almeida Prado Sampaio S, et al. The ultrastructural and histophotometric study of elastic and collagen fibers in type II Ehlers-Danlos syndrome and subclinical forms. Ultrastruct Pathol. 2000; 24: 129–34PubMedCrossRef
22.
Zurück zum Zitat Oxlung H. Relationships between the biomechanical properties, composition and molecular structure of connective tissues. Connect Tissue Res. 1986; 15: 65–72CrossRef Oxlung H. Relationships between the biomechanical properties, composition and molecular structure of connective tissues. Connect Tissue Res. 1986; 15: 65–72CrossRef
23.
Zurück zum Zitat Piérard GE, the EEMCO group. EEMCO guidance to the in vivo assessment of tensile functional properties of the skin: part 1. Relevance to the structures and ageing of the skin and subcutaneous tissues. Skin Pharmacol Appl Skin Physiol. 1999; 12: 352–62PubMedCrossRef Piérard GE, the EEMCO group. EEMCO guidance to the in vivo assessment of tensile functional properties of the skin: part 1. Relevance to the structures and ageing of the skin and subcutaneous tissues. Skin Pharmacol Appl Skin Physiol. 1999; 12: 352–62PubMedCrossRef
24.
Zurück zum Zitat Zweers MC, van Vlijmen-Willems IM, van Kuppevelt TH, et al. Deficiency of tenascin-X causes abnormalities in dermal elastic fiber morphology. J Invest Dermatol. 2004; 122: 885–91PubMedCrossRef Zweers MC, van Vlijmen-Willems IM, van Kuppevelt TH, et al. Deficiency of tenascin-X causes abnormalities in dermal elastic fiber morphology. J Invest Dermatol. 2004; 122: 885–91PubMedCrossRef
25.
Zurück zum Zitat Hermanns-Lê T, Piérard GE. Ultrastructural modifications of elastic fibers in the skin of Ehlers-Danlos syndrome hypermobile type. Am J Dermatopathol. In press. Hermanns-Lê T, Piérard GE. Ultrastructural modifications of elastic fibers in the skin of Ehlers-Danlos syndrome hypermobile type. Am J Dermatopathol. In press.
26.
Zurück zum Zitat Graham R, Beighton P. Physical properties of the skin in the Ehlers-Danlos syndrome. Ann Rheum Dis. 1969; 28: 246–51CrossRef Graham R, Beighton P. Physical properties of the skin in the Ehlers-Danlos syndrome. Ann Rheum Dis. 1969; 28: 246–51CrossRef
27.
Zurück zum Zitat Piérard GE, Lapiére ChM. Microanatomy of the dermis in relation to relaxed skin tension line and Langer’s lines. Am J Dermatopathol. 1987; 9: 219–24PubMedCrossRef Piérard GE, Lapiére ChM. Microanatomy of the dermis in relation to relaxed skin tension line and Langer’s lines. Am J Dermatopathol. 1987; 9: 219–24PubMedCrossRef
Metadaten
Titel
Collagen Fibril Arabesques in Connective Tissue Disorders
verfasst von
Trinh Hermanns-Lê
Prof. Gérald E. Piérard
Publikationsdatum
01.10.2006
Verlag
Springer International Publishing
Erschienen in
American Journal of Clinical Dermatology / Ausgabe 5/2006
Print ISSN: 1175-0561
Elektronische ISSN: 1179-1888
DOI
https://doi.org/10.2165/00128071-200607050-00006

Weitere Artikel der Ausgabe 5/2006

American Journal of Clinical Dermatology 5/2006 Zur Ausgabe

Review Article

Mycetoma

Therapy in Practice

Persistent Acne in Women

Leitlinien kompakt für die Dermatologie

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Studienlage spricht für Isotretinoin zur Rosazea-Therapie

23.05.2024 Rosazea Nachrichten

Isotretinoin wird off-label zur Behandlung von Rosazea eingesetzt. Wie solide die Evidenz dafür ist, wurde jetzt in einem systematischen Review überprüft.

So sicher sind Tattoos: Neue Daten zur Risikobewertung

22.05.2024 Melanom Nachrichten

Das größte medizinische Problem bei Tattoos bleiben allergische Reaktionen. Melanome werden dadurch offensichtlich nicht gefördert, die Farbpigmente könnten aber andere Tumoren begünstigen.

„Übersichtlicher Wegweiser“: Lauterbachs umstrittener Klinik-Atlas ist online

17.05.2024 Klinik aktuell Nachrichten

Sie sei „ethisch geboten“, meint Gesundheitsminister Karl Lauterbach: mehr Transparenz über die Qualität von Klinikbehandlungen. Um sie abzubilden, lässt er gegen den Widerstand vieler Länder einen virtuellen Klinik-Atlas freischalten.

Riesenzellarteriitis: 15% der Patienten sind von okkulter Form betroffen

16.05.2024 Riesenzellarteriitis Nachrichten

In einer retrospektiven Untersuchung haben Forschende aus Belgien und den Niederlanden die okkulte Form der Riesenzellarteriitis genauer unter die Lupe genommen. In puncto Therapie und Rezidivraten stellten sie keinen sehr großen Unterschied zu Erkrankten mit kranialen Symptomen fest.

Update Dermatologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.