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Acquired Cutis Laxa Concomitant With Nephrotic Syndrome

Acquired Cutis Laxa Concomitant With Nephrotic Syndrome Abstract • A 41-year-old woman developed laxity and wrinkling of the skin. This process spread slowly to involve the skin of almost the entire body, without any previous inflammatory skin disorder. The skin of her face, neck, and trunk hung in loose folds, leading to a prematurely aged appearance. One year later she noticed edema on her face and legs. Laboratory studies disclosed low C3 and CH50 serum levels and proteinuria. Skin biopsy specimens revealed extensive loss and fragmentation of dermal elastic fibers. A renal biopsy specimen showed membranoproliferative glomerulonephritis (type 2), and an immunofluorescent study disclosed C3 and IgG deposition in the mesangial matrix and along the glomerular basement membrane. We propose that, in this case, cutis laxa may be related to an abnormal immune response. (Arch Dermatol 1987;123:1211-1216) References 1. Harris RB, Heaphy MR, Perry HO: Generalized elastolysis (cutis laxa) . Am J Med 1978;65:815-822.Crossref 2. Kétly L: Ein Fall von eigenartiger Hautveränderung: `Chalodermie' (Schlaffhaut) . Arch Dermatol Syphilol Berlin 1901;56:107-121. 3. Goth A: Über Chalodermie (Kétly) . Dermatol Monatsschr 1937;104:426-435. 4. Carney RG, Nomland R: Acquired loose skin (chalazodermia): Report of a case . Arch Dermatol 1947;56:794-800. 5. Bettmann AG: Excessively relaxed skin and the pituitary gland . Plast Reconstr Surg 1955;15:489-501.Crossref 6. Engelke K: Zur Kasuistik der Dermatochalasis . Dtsch Gesundh 1957;12:272-273. 7. Marshall J, Vogelpoel L, Weber HW: Primary elastolysis . S Afr Med J 1960;34:721-727. 8. Schreiber MM, Tilley JC: Cutis laxa . Arch Dermatol 1961; 84:266-272.Crossref 9. McCarthy CF, Warin RP, Read AEA: Loose skin (cutis laxa) associated with systemic abnormalities . Arch Intern Med 1965;115:62-67.Crossref 10. Jablonska S: Inflammatorische Hautveränderungen, die einer Cutis Laxa Vorausgenen . Hautarzt 1966;17:341-346. 11. Shah BH, Sindhur CP, James MT: Cutis laxa . Indian J Dermatol 1968;34:152-157. 12. Wanderer AA, Ellis EF, Goltz RW, et al: Tracheobronchiomegaly and acquired cutis laxa . Pediatrics 1969;44:709-715. 13. Reed WB, Horowitz RE, Beighton P: Acquired cutis laxa: Primary generalized elastolysis . Arch Dermatol 1971;103:661-669.Crossref 14. Ferreira MC, Spina V: A case of cutis laxa with abnormal copper metabolism . Br J Plast Surg 1973;26:283-286.Crossref 15. Thaning O, Beighton P: Alimentary bleeding in cutis laxa of late onset . S Afr Med J 1972;46:928-930. 16. Hashimoto K, Kanzaki T: Cutis laxa: Ultrastructural and biochemical studies . Arch Dermatol 1975;111:861-873.Crossref 17. Kerl H, Burg G, Hashimoto K: Fatal, penicillin-induced, generalized, postinflammatory elastolysis (cutis laxa) . Am J Dermatopathol 1983;5:267-276.Crossref 18. Scott MA, Kauh YC, Luscombe HA: Acquired cutis laxa associated with multiple myeloma . Arch Dermatol 1976;112:853-855.Crossref 19. Nanko H, Jepsen LV, Zachariae H, et al: Acquired cutis laxa (generalized elastolysis): Light and electron microscopic studies . Acta Derm Venereol 1979;59:315-324. 20. Samantray SK: Cutis laxa . Postgrad Med 1981;69:65-68. 21. Muster AJ, Bharati S, Hermann JJ, et al: Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis . J Pediatr 1983;102:243-248.Crossref 22. Heilman VS, Haustein UF: Erworbene postinflammatorische Dermatochalasis nach Kälte-Urtikaria . Dermatol Monatsschr 1983;169:28-34. 23. Ting HC, Food MH, Wang F: Acquired cutis laxa and multiple myeloma . Br J Dermatol 1984;110:363-367.Crossref 24. Goltz RW, Hult AM, Goldfarb M, et al: Cutis laxa: A manifestation of generalized elastolysis . Arch Dermatol 1965; 92:373-387.Crossref 25. Byers PH, Siegel RC, Holbrook KA, et al: X-linked cutis laxa: Defective cross-link formation in collagen due to decreased lysyl oxidase activity . N Engl J Med 1980;303:61-65.Crossref 26. Brown FR, Holbrook KA, Byers PH, et al: Cutis laxa . Johns Hopkins Med J 1982;150:148-153. 27. Spitzer RE, Vallota EH, Forristal J, et al: Serum C3 lytic system in patients with glomerulonephritis . Science 1969;164:436-437.Crossref 28. Thompson RA: C3 inactivating factor in the serum of a patient with chronic hypocomplementaemic proliferative glomerulo-nephritis . Immunology 1972;22:147-158. 29. Peters DK, Martin A, Weinstein A, et al: Complement studies in membranoproliferative glomerulonephritis . Clin Exp Immunol 1972;11:311-320. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

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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.1987.01660330122024
Publisher site
See Article on Publisher Site

Abstract

Abstract • A 41-year-old woman developed laxity and wrinkling of the skin. This process spread slowly to involve the skin of almost the entire body, without any previous inflammatory skin disorder. The skin of her face, neck, and trunk hung in loose folds, leading to a prematurely aged appearance. One year later she noticed edema on her face and legs. Laboratory studies disclosed low C3 and CH50 serum levels and proteinuria. Skin biopsy specimens revealed extensive loss and fragmentation of dermal elastic fibers. A renal biopsy specimen showed membranoproliferative glomerulonephritis (type 2), and an immunofluorescent study disclosed C3 and IgG deposition in the mesangial matrix and along the glomerular basement membrane. We propose that, in this case, cutis laxa may be related to an abnormal immune response. (Arch Dermatol 1987;123:1211-1216) References 1. Harris RB, Heaphy MR, Perry HO: Generalized elastolysis (cutis laxa) . Am J Med 1978;65:815-822.Crossref 2. Kétly L: Ein Fall von eigenartiger Hautveränderung: `Chalodermie' (Schlaffhaut) . Arch Dermatol Syphilol Berlin 1901;56:107-121. 3. Goth A: Über Chalodermie (Kétly) . Dermatol Monatsschr 1937;104:426-435. 4. Carney RG, Nomland R: Acquired loose skin (chalazodermia): Report of a case . Arch Dermatol 1947;56:794-800. 5. Bettmann AG: Excessively relaxed skin and the pituitary gland . Plast Reconstr Surg 1955;15:489-501.Crossref 6. Engelke K: Zur Kasuistik der Dermatochalasis . Dtsch Gesundh 1957;12:272-273. 7. Marshall J, Vogelpoel L, Weber HW: Primary elastolysis . S Afr Med J 1960;34:721-727. 8. Schreiber MM, Tilley JC: Cutis laxa . Arch Dermatol 1961; 84:266-272.Crossref 9. McCarthy CF, Warin RP, Read AEA: Loose skin (cutis laxa) associated with systemic abnormalities . Arch Intern Med 1965;115:62-67.Crossref 10. Jablonska S: Inflammatorische Hautveränderungen, die einer Cutis Laxa Vorausgenen . Hautarzt 1966;17:341-346. 11. Shah BH, Sindhur CP, James MT: Cutis laxa . Indian J Dermatol 1968;34:152-157. 12. Wanderer AA, Ellis EF, Goltz RW, et al: Tracheobronchiomegaly and acquired cutis laxa . Pediatrics 1969;44:709-715. 13. Reed WB, Horowitz RE, Beighton P: Acquired cutis laxa: Primary generalized elastolysis . Arch Dermatol 1971;103:661-669.Crossref 14. Ferreira MC, Spina V: A case of cutis laxa with abnormal copper metabolism . Br J Plast Surg 1973;26:283-286.Crossref 15. Thaning O, Beighton P: Alimentary bleeding in cutis laxa of late onset . S Afr Med J 1972;46:928-930. 16. Hashimoto K, Kanzaki T: Cutis laxa: Ultrastructural and biochemical studies . Arch Dermatol 1975;111:861-873.Crossref 17. Kerl H, Burg G, Hashimoto K: Fatal, penicillin-induced, generalized, postinflammatory elastolysis (cutis laxa) . Am J Dermatopathol 1983;5:267-276.Crossref 18. Scott MA, Kauh YC, Luscombe HA: Acquired cutis laxa associated with multiple myeloma . Arch Dermatol 1976;112:853-855.Crossref 19. Nanko H, Jepsen LV, Zachariae H, et al: Acquired cutis laxa (generalized elastolysis): Light and electron microscopic studies . Acta Derm Venereol 1979;59:315-324. 20. Samantray SK: Cutis laxa . Postgrad Med 1981;69:65-68. 21. Muster AJ, Bharati S, Hermann JJ, et al: Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis . J Pediatr 1983;102:243-248.Crossref 22. Heilman VS, Haustein UF: Erworbene postinflammatorische Dermatochalasis nach Kälte-Urtikaria . Dermatol Monatsschr 1983;169:28-34. 23. Ting HC, Food MH, Wang F: Acquired cutis laxa and multiple myeloma . Br J Dermatol 1984;110:363-367.Crossref 24. Goltz RW, Hult AM, Goldfarb M, et al: Cutis laxa: A manifestation of generalized elastolysis . Arch Dermatol 1965; 92:373-387.Crossref 25. Byers PH, Siegel RC, Holbrook KA, et al: X-linked cutis laxa: Defective cross-link formation in collagen due to decreased lysyl oxidase activity . N Engl J Med 1980;303:61-65.Crossref 26. Brown FR, Holbrook KA, Byers PH, et al: Cutis laxa . Johns Hopkins Med J 1982;150:148-153. 27. Spitzer RE, Vallota EH, Forristal J, et al: Serum C3 lytic system in patients with glomerulonephritis . Science 1969;164:436-437.Crossref 28. Thompson RA: C3 inactivating factor in the serum of a patient with chronic hypocomplementaemic proliferative glomerulo-nephritis . Immunology 1972;22:147-158. 29. Peters DK, Martin A, Weinstein A, et al: Complement studies in membranoproliferative glomerulonephritis . Clin Exp Immunol 1972;11:311-320.

Journal

Archives of DermatologyAmerican Medical Association

Published: Sep 1, 1987

References