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Osteopoikilosis Associated With Dermatofibrosis Lenticularis Disseminata

Osteopoikilosis Associated With Dermatofibrosis Lenticularis Disseminata Abstract We report a patient with osteopoikilosis and dermatofibrosis lenticularis disseminata with unusual flexural location. The patient was undersized, had diabetes mellitus, and pronounced arteriosclerotic changes. Histologic examination showed the cutaneous lesions sharply demarcated with a pseudotumorous appearance. Electron microscopic studies of these lesions revealed some of the elastic fibers broken up, partly into big lumps consisting of the amorphous component, partly into smaller granular elements, presumably representing the fibrillar component. The collagen fibers were normal. A certain resemblance to pseudoxanthoma elasticum is discussed. References 1. Albers-Schönberg, H.: Eine Seltene, Bisher Nicht Bekannte Strukturanomalie des Skelettes , Fortschr Roentgenstr 23:174-175 ( (July) ) 1915. 2. Windholz, F.: Über familiäre Osteopoikilie und Dermatofibrosis Lenticularis Disseminata , Fortschr Roentgenstr 45:566-582 ( (May) ) 1932. 3. Busch, K.F.B.: Osteosclerosis Disseminata Familiaris, thesis, Hasselbalch, Copenhagen, 1936. 4. Berlin, R., et al: Osteopoikilosis: A Clinical and Genetic Study , Acta Med Scand 181:305-314 ( (March) ) 1967.Crossref 5. Schmorl, G.: Anatomische Befunde bei Einem Falle von Osteopoikilie , Fortschr Roentgenstr 44:1-8 ( (July) ) 1931. 6. Buschke, A., and Ollendorff, H.: Ein Fall von Dermatofibrosis Lenticularis Disseminata und Osteopathia Condensans Disseminata , Derm Wschr 86:257-262 ( (Feb 25) ) 1928. 7. Bethge, J.F.J., and Ridderbusch, K.E.: Über Osteopoikilie und das Neue Krankheitsbild Hyperostose bei Osteopoikikie , Ergebn Chir Orthop 49:138-182, 1967. 8. Serowy, C.: Ein Beitrag zum Syndrom Dermatofibrosis Lenticularis Disseminata und Osteopoikilie , Arch Klin Exp Derm 203:113-124 ( (June) ) 1956.Crossref 9. Smith, C.A.D., and Waisman, M.: Connective Tissue Nevi: Familial Occurrence and Association With Osteopoikilosis , Arch Derm 81:249-252 ( (Feb) ) 1960.Crossref 10. McKusick, V.A.: Heritable Disorders of Connective Tissue , ed 3, St. Louis: C. V. Mosby Company, 1966, pp 286-324. 11. Midtgård, K.: Connective Tissue in Diabetes: An Electron Microscopic Investigation to be published. 12. Danielsen, L., et al: Pseudoxanthoma Elasticum: A Clinical, Ophthalmological and Electron Microscopic Study, to be published. 13. Svab, V.: A Propos de L'Osteopaecilie Héréditaire , J Radiol Electr 16:405-415 ( (Sept) ) 1932. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Osteopoikilosis Associated With Dermatofibrosis Lenticularis Disseminata

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References (10)

Publisher
American Medical Association
Copyright
Copyright © 1969 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1969.01610280083016
Publisher site
See Article on Publisher Site

Abstract

Abstract We report a patient with osteopoikilosis and dermatofibrosis lenticularis disseminata with unusual flexural location. The patient was undersized, had diabetes mellitus, and pronounced arteriosclerotic changes. Histologic examination showed the cutaneous lesions sharply demarcated with a pseudotumorous appearance. Electron microscopic studies of these lesions revealed some of the elastic fibers broken up, partly into big lumps consisting of the amorphous component, partly into smaller granular elements, presumably representing the fibrillar component. The collagen fibers were normal. A certain resemblance to pseudoxanthoma elasticum is discussed. References 1. Albers-Schönberg, H.: Eine Seltene, Bisher Nicht Bekannte Strukturanomalie des Skelettes , Fortschr Roentgenstr 23:174-175 ( (July) ) 1915. 2. Windholz, F.: Über familiäre Osteopoikilie und Dermatofibrosis Lenticularis Disseminata , Fortschr Roentgenstr 45:566-582 ( (May) ) 1932. 3. Busch, K.F.B.: Osteosclerosis Disseminata Familiaris, thesis, Hasselbalch, Copenhagen, 1936. 4. Berlin, R., et al: Osteopoikilosis: A Clinical and Genetic Study , Acta Med Scand 181:305-314 ( (March) ) 1967.Crossref 5. Schmorl, G.: Anatomische Befunde bei Einem Falle von Osteopoikilie , Fortschr Roentgenstr 44:1-8 ( (July) ) 1931. 6. Buschke, A., and Ollendorff, H.: Ein Fall von Dermatofibrosis Lenticularis Disseminata und Osteopathia Condensans Disseminata , Derm Wschr 86:257-262 ( (Feb 25) ) 1928. 7. Bethge, J.F.J., and Ridderbusch, K.E.: Über Osteopoikilie und das Neue Krankheitsbild Hyperostose bei Osteopoikikie , Ergebn Chir Orthop 49:138-182, 1967. 8. Serowy, C.: Ein Beitrag zum Syndrom Dermatofibrosis Lenticularis Disseminata und Osteopoikilie , Arch Klin Exp Derm 203:113-124 ( (June) ) 1956.Crossref 9. Smith, C.A.D., and Waisman, M.: Connective Tissue Nevi: Familial Occurrence and Association With Osteopoikilosis , Arch Derm 81:249-252 ( (Feb) ) 1960.Crossref 10. McKusick, V.A.: Heritable Disorders of Connective Tissue , ed 3, St. Louis: C. V. Mosby Company, 1966, pp 286-324. 11. Midtgård, K.: Connective Tissue in Diabetes: An Electron Microscopic Investigation to be published. 12. Danielsen, L., et al: Pseudoxanthoma Elasticum: A Clinical, Ophthalmological and Electron Microscopic Study, to be published. 13. Svab, V.: A Propos de L'Osteopaecilie Héréditaire , J Radiol Electr 16:405-415 ( (Sept) ) 1932.

Journal

Archives of DermatologyAmerican Medical Association

Published: Oct 1, 1969

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