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Ehlers-Danlos Syndrome: Correlation of Clinical and Histopathological Findings

Ehlers-Danlos Syndrome: Correlation of Clinical and Histopathological Findings Abstract Ehlers-Danlos' syndrome is a dysplasia of the mesenchymal tissue characterized by abnormal hyperlaxity and elasticity of the skin; dermofragility, or easy bruising and splitting of the skin at the slightest provocation, with formation of deep wounds, hematomas, and pseudotumors, and, finally, hyperlaxity of the joints. Tobias10 and others8 suggested that the symptoms were manifestations of a generalized mesenchymal anomaly with changes in the connective tissue in skin, tendons, joint capsules, large vessels, and internal structures. Publications based on necropsy findings in Ehlers-Danlos' syndrome have not been found in the literature, and definite proof of a universal mesenchymal anomaly is lacking. Tscherno-Gubow first described the condition in 1892, whereas Ehlers4 made his report in 1901 and Danlos,3 in 1908, respectively. Most cases occur in isolation,1,3-5,7,8,10,12, 17-19 and some authors consider the condition a recessive one. Others, as Wiener12 and References 1. Brown, A., and Stock, V. F.: Dermatorrhexis: Report of a Case , Am. J. Dis. Child. 54:956, 1937. 2. Danbolt, N.: Cutis Hyperelastica: Ehlers-Danlos Syndrome, Acta dermat.-venereol. 3. Danlos, H. A., cited in Reference 10. 4. Ehlers, E.: Cutis Laxa: Neigung zu Haemorrhagien in der Haut., Lockerung mehrerer Artikulationen , Dermat. Ztschr. 8:173, 1901. 5. Haxthausen, H.: Cutis Laxa , Acta dermat.-venereol. 17:601, 1936. 6. Johnson, St. A. M., and Falls, H. F.: Ehlers-Danlos Syndrome: A Clinical and Genetic Study , Arch. Dermat. & Syph. 60:82, 1949. 7. Ronchese, F.: Dermatorrhexis with Dermatochalasis and Arthrochalasis (the So-Called Ehlers-Danlos Syndrome) , Am. J. Dis. Child. 51:1403, 1936. 8. Schoch, A.: Ehlers-Danlos'sches Syndrom , Dermatologica 92:290, 1946. 9. Strandberg, J.: Cutis Laxa , Nord. med. 1:626, 1939. 10. Tobias, N.: Danlos Syndrome Associated with Congenital Lipomatosis , Arch. Dermat. & Syph. 30:540, 1934. 11. Tobias, N.: Danlos Syndrome , Arch. Dermat. & Syph. 40:137, 1939. 12. Wiener, K.: Gummihaut (Cutis Laxa) mit dominanter Vererbung , Arch. Dermat. u. Syph. 148:599, 1925. 13. Wigers, F.: Ehlers-Danlos Syndrome: Cutis Hyperelastica , Nord. med. 43:304, 1950. 14. Husebye, K. O.: Ehlers-Danlos syndrom: Omtale av tre tilfelle i en sterkt belastet slekt , Tidsskr. norske laegefor. 72:185, 1952. 15. Ormsby, O. S., and Montgomery, H.: Diseases of the Skin , Philadelphia, Lea & Febiger, 1954. 16. Lewitus, Z.: Ehlers-Danlos Syndrome: Report of 2 Cases with Hypophyseal Dysfunction , A. M. A. Arch. Dermat. 73:158, 1956. 17. Weber, F. P., and Aitkin, J. K.: Nature of the Subcutaneous Spherules in Some Cases of the Ehlers-Danlos' Syndrome , Lancet 1:198, 1938. 18. Bettman, A. G.: Excessively Relaxed Skin and the Pituitary Gland , Plast. & Reconstruct. Surg. 15:6, 1955. 19. King-Lewis, F., and Polunin, I. V.: Two Cases of Ehlers-Danlos Syndrome , Arch. Dis. Childhood 22:170, 1947. 20. Jacobs, P. H.: Ehlers-Danlos Syndrome: Report of a Case with Onset at Age 29 , A. M. A. Arch. Dermat. 76:460, 1957. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Dermatology American Medical Association

Ehlers-Danlos Syndrome: Correlation of Clinical and Histopathological Findings

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References (22)

Publisher
American Medical Association
Copyright
Copyright © 1958 American Medical Association. All Rights Reserved.
ISSN
0096-5359
DOI
10.1001/archderm.1958.01560120052008
Publisher site
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Abstract

Abstract Ehlers-Danlos' syndrome is a dysplasia of the mesenchymal tissue characterized by abnormal hyperlaxity and elasticity of the skin; dermofragility, or easy bruising and splitting of the skin at the slightest provocation, with formation of deep wounds, hematomas, and pseudotumors, and, finally, hyperlaxity of the joints. Tobias10 and others8 suggested that the symptoms were manifestations of a generalized mesenchymal anomaly with changes in the connective tissue in skin, tendons, joint capsules, large vessels, and internal structures. Publications based on necropsy findings in Ehlers-Danlos' syndrome have not been found in the literature, and definite proof of a universal mesenchymal anomaly is lacking. Tscherno-Gubow first described the condition in 1892, whereas Ehlers4 made his report in 1901 and Danlos,3 in 1908, respectively. Most cases occur in isolation,1,3-5,7,8,10,12, 17-19 and some authors consider the condition a recessive one. Others, as Wiener12 and References 1. Brown, A., and Stock, V. F.: Dermatorrhexis: Report of a Case , Am. J. Dis. Child. 54:956, 1937. 2. Danbolt, N.: Cutis Hyperelastica: Ehlers-Danlos Syndrome, Acta dermat.-venereol. 3. Danlos, H. A., cited in Reference 10. 4. Ehlers, E.: Cutis Laxa: Neigung zu Haemorrhagien in der Haut., Lockerung mehrerer Artikulationen , Dermat. Ztschr. 8:173, 1901. 5. Haxthausen, H.: Cutis Laxa , Acta dermat.-venereol. 17:601, 1936. 6. Johnson, St. A. M., and Falls, H. F.: Ehlers-Danlos Syndrome: A Clinical and Genetic Study , Arch. Dermat. & Syph. 60:82, 1949. 7. Ronchese, F.: Dermatorrhexis with Dermatochalasis and Arthrochalasis (the So-Called Ehlers-Danlos Syndrome) , Am. J. Dis. Child. 51:1403, 1936. 8. Schoch, A.: Ehlers-Danlos'sches Syndrom , Dermatologica 92:290, 1946. 9. Strandberg, J.: Cutis Laxa , Nord. med. 1:626, 1939. 10. Tobias, N.: Danlos Syndrome Associated with Congenital Lipomatosis , Arch. Dermat. & Syph. 30:540, 1934. 11. Tobias, N.: Danlos Syndrome , Arch. Dermat. & Syph. 40:137, 1939. 12. Wiener, K.: Gummihaut (Cutis Laxa) mit dominanter Vererbung , Arch. Dermat. u. Syph. 148:599, 1925. 13. Wigers, F.: Ehlers-Danlos Syndrome: Cutis Hyperelastica , Nord. med. 43:304, 1950. 14. Husebye, K. O.: Ehlers-Danlos syndrom: Omtale av tre tilfelle i en sterkt belastet slekt , Tidsskr. norske laegefor. 72:185, 1952. 15. Ormsby, O. S., and Montgomery, H.: Diseases of the Skin , Philadelphia, Lea & Febiger, 1954. 16. Lewitus, Z.: Ehlers-Danlos Syndrome: Report of 2 Cases with Hypophyseal Dysfunction , A. M. A. Arch. Dermat. 73:158, 1956. 17. Weber, F. P., and Aitkin, J. K.: Nature of the Subcutaneous Spherules in Some Cases of the Ehlers-Danlos' Syndrome , Lancet 1:198, 1938. 18. Bettman, A. G.: Excessively Relaxed Skin and the Pituitary Gland , Plast. & Reconstruct. Surg. 15:6, 1955. 19. King-Lewis, F., and Polunin, I. V.: Two Cases of Ehlers-Danlos Syndrome , Arch. Dis. Childhood 22:170, 1947. 20. Jacobs, P. H.: Ehlers-Danlos Syndrome: Report of a Case with Onset at Age 29 , A. M. A. Arch. Dermat. 76:460, 1957.

Journal

A.M.A. Archives of Dermatology – American Medical Association

Published: Dec 1, 1958

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