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An Ultrastructural Study of the Reactive Type of Elastosis Perforans Serpiginosa

An Ultrastructural Study of the Reactive Type of Elastosis Perforans Serpiginosa Abstract To the Editor.— Since elastosis perforans serpiginosa (EPS) was first described, it has been suggested that it is a phenomenon caused by a variety of abnormal elastic fibers. At least three etiologic types are now considered to be involved, ie, the idiopathic, the reactive, and the penicillamine-induced.1-3 Previous ultrastructural investigations showed that the abnormal elastic fibers in the idiopathic type differ in structure from those of the penicillamine-induced type.1,3-6 The reactive type, which is associated with different congenital disorders, mostly of the connective tissue, such as Down's syndrome, osteogenesis imperfecta, Marfan's syndrome, pseudoxanthoma elasticum, and Ehlers-Danlos syndrome (EDS), has not been as extensively studied.7 It remains to be demonstrated whether it is a unique structural form of EPS.We performed an ultrastructural study in two patients with the reactive type of EPS. Report of Cases.—Case 1.— A 15-year-old girl presented with osteogenesis imperfecta, type Ia, References 1. Bardach H, Gebhart W, Niebauer G: `Lumpy-bumpy' elastic fibers in the skin and lungs of a patient with a penicillamineinduced elastosis perforans serpiginosa . J Cutan Pathol 1979; 6:243-252.Crossref 2. Patterson JW: Perforating disorders . J Am Acad Dermatol 1984;10:561-581.Crossref 3. Hashimoto K, McEvoy B, Belcher R: Ultrastructure of penicillamine-induced skin lesions . J Am Acad Dermatol 1981;4:300-315.Crossref 4. Cohen AS, Hashimoto K: Electron microscopic observations on the lesions of elastosis perforans serpiginosa . J Invest Dermatol 1960;35:15-19.Crossref 5. Volpin D, Pasquali-Ronchetti I, Castellani I, et al: Ultrastructural and biochemical studies on a case of elastosis perforans serpiginosa . Dermatologica 1978;156:209-223.Crossref 6. Kirsch N, Hukill PB: Elastosis perforans serpiginosa induced by penicillamine . Arch Dermatol 1977;113:630-635.Crossref 7. Crotty G, Bell M, Estes SA, et al: Cytologic features of elastosis perforans serpiginosa (EPS) associated with Down's syndrome . J Am Acad Dermatol 1983;8:255-256.Crossref 8. Pinnell SR, Murad S: Disorders of collagen , in Stansbury JB, Wyngaarden JB, Fredrickson DS, et al (eds): The Metabolic Basis of Inherited Disease . New York, McGraw-Hill International Book Co, 1983, pp 1434-1441. 9. Prockop DJ, Kivirikko KI: Heritable diseases of collagen . N Engl J Med 1984;311:376-386.Crossref 10. Holbrook KA, Byers PH: Structural abnormalities in the dermal collagen and elastic matrix of patients with inherited connective tissue disorders . J Invest Dermatol 1982;79:7s-16s.Crossref 11. Bergman R, Friedman-Birnbaum R, Hazaz B: A direct immunofluorescence study in elastosis perforans serpiginosa . Br J Dermatol 1985;113:573-579.Crossref 12. Werb Z, Banda MJ, McKerrow JH, et al: Elastases and elastin degradation . J Invest Dermatol 1982;79:154s-159s.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

An Ultrastructural Study of the Reactive Type of Elastosis Perforans Serpiginosa

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Publisher
American Medical Association
Copyright
Copyright © 1987 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1987.01660330034007
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.— Since elastosis perforans serpiginosa (EPS) was first described, it has been suggested that it is a phenomenon caused by a variety of abnormal elastic fibers. At least three etiologic types are now considered to be involved, ie, the idiopathic, the reactive, and the penicillamine-induced.1-3 Previous ultrastructural investigations showed that the abnormal elastic fibers in the idiopathic type differ in structure from those of the penicillamine-induced type.1,3-6 The reactive type, which is associated with different congenital disorders, mostly of the connective tissue, such as Down's syndrome, osteogenesis imperfecta, Marfan's syndrome, pseudoxanthoma elasticum, and Ehlers-Danlos syndrome (EDS), has not been as extensively studied.7 It remains to be demonstrated whether it is a unique structural form of EPS.We performed an ultrastructural study in two patients with the reactive type of EPS. Report of Cases.—Case 1.— A 15-year-old girl presented with osteogenesis imperfecta, type Ia, References 1. Bardach H, Gebhart W, Niebauer G: `Lumpy-bumpy' elastic fibers in the skin and lungs of a patient with a penicillamineinduced elastosis perforans serpiginosa . J Cutan Pathol 1979; 6:243-252.Crossref 2. Patterson JW: Perforating disorders . J Am Acad Dermatol 1984;10:561-581.Crossref 3. Hashimoto K, McEvoy B, Belcher R: Ultrastructure of penicillamine-induced skin lesions . J Am Acad Dermatol 1981;4:300-315.Crossref 4. Cohen AS, Hashimoto K: Electron microscopic observations on the lesions of elastosis perforans serpiginosa . J Invest Dermatol 1960;35:15-19.Crossref 5. Volpin D, Pasquali-Ronchetti I, Castellani I, et al: Ultrastructural and biochemical studies on a case of elastosis perforans serpiginosa . Dermatologica 1978;156:209-223.Crossref 6. Kirsch N, Hukill PB: Elastosis perforans serpiginosa induced by penicillamine . Arch Dermatol 1977;113:630-635.Crossref 7. Crotty G, Bell M, Estes SA, et al: Cytologic features of elastosis perforans serpiginosa (EPS) associated with Down's syndrome . J Am Acad Dermatol 1983;8:255-256.Crossref 8. Pinnell SR, Murad S: Disorders of collagen , in Stansbury JB, Wyngaarden JB, Fredrickson DS, et al (eds): The Metabolic Basis of Inherited Disease . New York, McGraw-Hill International Book Co, 1983, pp 1434-1441. 9. Prockop DJ, Kivirikko KI: Heritable diseases of collagen . N Engl J Med 1984;311:376-386.Crossref 10. Holbrook KA, Byers PH: Structural abnormalities in the dermal collagen and elastic matrix of patients with inherited connective tissue disorders . J Invest Dermatol 1982;79:7s-16s.Crossref 11. Bergman R, Friedman-Birnbaum R, Hazaz B: A direct immunofluorescence study in elastosis perforans serpiginosa . Br J Dermatol 1985;113:573-579.Crossref 12. Werb Z, Banda MJ, McKerrow JH, et al: Elastases and elastin degradation . J Invest Dermatol 1982;79:154s-159s.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Sep 1, 1987

References