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Cutis Laxa: Ultrastructural and Biochemical Studies

Cutis Laxa: Ultrastructural and Biochemical Studies Abstract Specimens from one case each of acquired and congenital cutis laxa were examined by electron microscopy. Elastic fibers of the dermis and subcutaneous arteries and veins were found to have similar changes. Elastin was diminished and microfilaments were visible throughout the entire fiber. Electron-dense, amorphous or granular layers that alternate with relatively electron-light layers were deficient. This electron-dense substance was unevenly aggregated within or in the vicinity of elastic fibers. In the congenital case, the aggregation and deposition of the electron-dense substance was often pronounced. In the vein, deficient deposition of elastin and admixture of microfilaments and amorphous substance were also found. Such admixture was often encircled by fibroblasts or smooth muscle cells. In the artery, multiplication of basal lamina was seen. Collagen fibers and anchoring fibrils of the skin were normal. References 1. Rook A: Disorders of connective tissue , in Rook A, Wilkinson DS, Ebling FJG (eds): Textbook of Dermatology , ed 2. Beccles, England, William Clowes & Sons, 1972, pp 1468-1469. 2. Hult AM, Goltz RW, Midtgaard K: The dermal elastic fibers in cutis hyperelastica (Ehlers-Danlos syndrome) and in cutis laxa (generalized elastolysis): A histological, histochemical, and electron microscopic study . Acta Derm Venereol 44:415-420, 1964. 3. Goltz RW, Hult AM, Goldfarb M, et al: Cutis laxa: A manifestation of generalized elastolysis . Arch Dermatol 92:373-387, 1965.Crossref 4. McCarthy CF, Warin RP, Read AEA: Loose skin (cutis laxa) associated with systemic abnormalities . Arch Intern Med 115:62-67, 1965.Crossref 5. Eriksson S: Studies in α1-antitrypsin deficiency . Acta Med Scand Suppl 432:1-85, 1965. 6. Kanzaki T, Hashimoto K: Acquired cutis laxa, biochemical and electron microscopic studies , abstracted. Clin Res 21:478, 1973. 7. Dermatochalasia (dermatomegaly) and congenital pulmonic stenosis, society transactions . Arch Dermatol 97:602-603, 1968.Crossref 8. Reynolds ES: The use of lead citrate at high pH as an electron opaque stain in electron microscopy . J Cell Biol 17:208-212, 1963.Crossref 9. Hashimoto K: Ultrastructure of freezecleaved dermal collagen . Acta Derm Venereol , to be published. 10. Ross R, Bornstein P: The elastic fiber: I. The separation and partial characterization of its macromolecular components . J Cell Biol 40:366, 1969.Crossref 11. Ross R, Bornstein P: Studies of the components of the elastic fiber , in Balazs EA (ed): Chemistry and Molecular Biology of the Intercellular Matrix . New York, Academic Press Inc, 1970, pp 641-655. 12. Varadi DP: Studies on the chemistry and fine structure of elastic fibers from normal adult skin . J Invest Dermatol 59:238-246, 1972.Crossref 13. Hashimoto K, Dibella RJ: Electron microscopic studies of normal and abnormal elastic fibers of the skin . J Invest Dermatol 48:405-423, 1967. 14. Hashimoto K, Lever WF: Appendage Tumors of the Skin . Springfield, Ill, Charles C Thomas Publishers, 1968, p 164. 15. Hashimoto K, Yamanishi Y, Maeyens E, et al: Collagenolytic activities of squamous cell carcinoma of the skin . Cancer Res 33:2790-2801, 1973. 16. Hashimoto K, Ohyama H: Cross-banded filamentous aggregation in the human dermis . J Invest Dermatol 62:106-112, 1974.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Cutis Laxa: Ultrastructural and Biochemical Studies

Archives of Dermatology , Volume 111 (7) – Jul 1, 1975

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Publisher
American Medical Association
Copyright
Copyright © 1975 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1975.01630190051004
Publisher site
See Article on Publisher Site

Abstract

Abstract Specimens from one case each of acquired and congenital cutis laxa were examined by electron microscopy. Elastic fibers of the dermis and subcutaneous arteries and veins were found to have similar changes. Elastin was diminished and microfilaments were visible throughout the entire fiber. Electron-dense, amorphous or granular layers that alternate with relatively electron-light layers were deficient. This electron-dense substance was unevenly aggregated within or in the vicinity of elastic fibers. In the congenital case, the aggregation and deposition of the electron-dense substance was often pronounced. In the vein, deficient deposition of elastin and admixture of microfilaments and amorphous substance were also found. Such admixture was often encircled by fibroblasts or smooth muscle cells. In the artery, multiplication of basal lamina was seen. Collagen fibers and anchoring fibrils of the skin were normal. References 1. Rook A: Disorders of connective tissue , in Rook A, Wilkinson DS, Ebling FJG (eds): Textbook of Dermatology , ed 2. Beccles, England, William Clowes & Sons, 1972, pp 1468-1469. 2. Hult AM, Goltz RW, Midtgaard K: The dermal elastic fibers in cutis hyperelastica (Ehlers-Danlos syndrome) and in cutis laxa (generalized elastolysis): A histological, histochemical, and electron microscopic study . Acta Derm Venereol 44:415-420, 1964. 3. Goltz RW, Hult AM, Goldfarb M, et al: Cutis laxa: A manifestation of generalized elastolysis . Arch Dermatol 92:373-387, 1965.Crossref 4. McCarthy CF, Warin RP, Read AEA: Loose skin (cutis laxa) associated with systemic abnormalities . Arch Intern Med 115:62-67, 1965.Crossref 5. Eriksson S: Studies in α1-antitrypsin deficiency . Acta Med Scand Suppl 432:1-85, 1965. 6. Kanzaki T, Hashimoto K: Acquired cutis laxa, biochemical and electron microscopic studies , abstracted. Clin Res 21:478, 1973. 7. Dermatochalasia (dermatomegaly) and congenital pulmonic stenosis, society transactions . Arch Dermatol 97:602-603, 1968.Crossref 8. Reynolds ES: The use of lead citrate at high pH as an electron opaque stain in electron microscopy . J Cell Biol 17:208-212, 1963.Crossref 9. Hashimoto K: Ultrastructure of freezecleaved dermal collagen . Acta Derm Venereol , to be published. 10. Ross R, Bornstein P: The elastic fiber: I. The separation and partial characterization of its macromolecular components . J Cell Biol 40:366, 1969.Crossref 11. Ross R, Bornstein P: Studies of the components of the elastic fiber , in Balazs EA (ed): Chemistry and Molecular Biology of the Intercellular Matrix . New York, Academic Press Inc, 1970, pp 641-655. 12. Varadi DP: Studies on the chemistry and fine structure of elastic fibers from normal adult skin . J Invest Dermatol 59:238-246, 1972.Crossref 13. Hashimoto K, Dibella RJ: Electron microscopic studies of normal and abnormal elastic fibers of the skin . J Invest Dermatol 48:405-423, 1967. 14. Hashimoto K, Lever WF: Appendage Tumors of the Skin . Springfield, Ill, Charles C Thomas Publishers, 1968, p 164. 15. Hashimoto K, Yamanishi Y, Maeyens E, et al: Collagenolytic activities of squamous cell carcinoma of the skin . Cancer Res 33:2790-2801, 1973. 16. Hashimoto K, Ohyama H: Cross-banded filamentous aggregation in the human dermis . J Invest Dermatol 62:106-112, 1974.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Jul 1, 1975

References

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