Rheumaderm pp 15-21 | Cite as

Heritable Connective Tissue Disorders

  • Jouni UittoEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 455)


The extracellular matrix of connective tissue of the skin consists of a large number of distinct matrix macromolecules which play a critical role in providing physiologic properties to normal skin. Within the dermis, collagen and elastic fibers are the two major fibrillar components of connective tissue, and they exist in a fibrous interwoven mesh- work structure associated with other extracellular macromolecules of the dermis, such as proteoglycan/glycosaminoglycan complexes, and a number of non-collagenous glycoproteins, including fibronectin and fibrillins (Tables 1 and 2). Encased in the fibrillar mesh- work are cells that interact with the extracellular matrix components through cell-matrix receptors, such as integrins.


Elastic Fiber Marfan Syndrome Bullous Pemphigoid Epidermolysis Bullosa Pulmonary Emphysema 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Selected Reviews

  1. Uitto, J., and Fazio, M. Biology and Pathophysiology of the Extracellular Matrix in Dermis. In: Pathophysiology of Dermatologic Diseases (Soter, N.A., and Baden, H.P., eds), 2nd Edition, McGraw-Hill, New York, pp.349–367, 1990.Google Scholar
  2. Uitto, J., Fazio, M.J., and Olsen, D.R. Heritable Disorders of Connective Tissue. In: Genetic Disorders of the Skin (Alper, J.C., ed), Mosby-Year Book, Inc., St. Louis, MO, pp. 287–319, 1991.Google Scholar
  3. Uitto, J., McGrath, J., and Bernstein, E. The Dermis. In: Cutaneous Medicine and Surgery: An Integrated Program in Dermatology (K.A. Arndt, P.E. LeBoit, J.K. Robinson, and B.U. Wintroub, eds), W.B. Saunders Company, Philadelphia, PA, vol. 1, pp. 857–881, 1996.Google Scholar
  4. Rosenbloom, J. The Elastic Fiber in Health and Disease. Progress in Dermatology, vol. 30(4), pp. 1–15, 1996.Google Scholar

Copyright information

© Springer Science+Business Media New York 1999

Authors and Affiliations

  1. 1.Department of Dermatology and Cutaneous BiologyJefferson Medical CollegePhiladelphiaUSA

Personalised recommendations