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Sweet's Syndrome Leading to Acquired Cutis Laxa (Marshall's Syndrome) in an Infant With a1-Antitrypsin Deficiency

Sweet's Syndrome Leading to Acquired Cutis Laxa (Marshall's Syndrome) in an Infant With... Abstract Background: Marshall's syndrome is a rare pediatric skin disease that is characterized by acquired, localized neutrophilic dermatitis (Sweet's disease), followed by loss of elastic tissue in the dermis and cutis laxa. The cause of this syndrome is unknown. α1-Antitrypsin (α1-AT) deficiency is a codominantly inherited disorder of α1-AT, the major serum antiprotease active against a number of serine-type proteases. Observations: The first patient with classic Marshall's syndrome who had coexisting α1-AT deficiency and a review of other cases of Marshall's syndrome are pre- sented, and pathogenic mechanisms are discussed. Conclusions: A deficiency of α1-AT may allow proteases such as neutrophil elastase to destroy dermal elastin and, thus, produce cutis laxa in Marshall's syndrome. Other cases of acquired cutis laxa should be screened for α1-AT deficiency to further evaluate this association and to enable patients and their families to be counseled about possible systemic complications of α1-AT deficiency.(Arch Dermatol. 1995;131:1175-1177) References 1. Lewis F, Lewis-Jones S, Gipson M. Acquired cutis laxa with dermatitis herpetiformis and sarcoidosis . J Am Acad Dermatol. 1993;29:846-848.Crossref 2. Kerl H, Burg G, Hashimoto K. Fatal penicillin induced generalised post-inflammatory elastolysis (cutis laxa) . Am J Dermatopathol. 1983;3:267-276.Crossref 3. Marshall J, Heyl T, Weber HW. Postinflammatory elastolysis and cutis laxa . S Afr Med J. 1966;40:1016-1022. 4. Sweet R. An acute febrile neutrophilic dermatosis . Br J Dermatol. 1964;76: 349-356.Crossref 5. Koch S, Williams M. Acquired cutis laxa . Pediatr Dermatol. 1985;2:282-288.Crossref 6. Kemmett D, Hunter J. Sweet's syndrome . J Am Acad Dermatol. 1990;23:503-507.Crossref 7. Petrozzi J, Wartham T. Sweet's syndrome: unique local response to streptococcal antigen . Cutis. 1971;84:393-399. 8. Saxe N, Gordon W. Acute febrile neutrophilic dermatosis (Sweet's syndrome): four case reports . S Afr Med J. 1978;52:253-256. 9. Reed W, Horowitz R, Beighton P. Acquired cutis laxa: primary generalized elastolysis . Arch Dermatol. 1971;103:661-669.Crossref 10. Goltz R, Hult A, Goldfarb M, et al. Cutis laxa: a manifestation of generalized elastolysis . Arch Dermatol. 1965;92:373-387.Crossref 11. Wulfsberg EA, Hoffman DE, Cohen MM. Alpha-1-antitrypsin deficiency: impact of genetic discovery on medicine and society . JAMA. 1994;271:217-222.Crossref 12. Perlmutter D. The cellular basis for liver injury in alpha-1-antitrypsin deficiency . Hepatology. 1991;13:172-185.Crossref 13. Smith KC, Su WP, Pittelkow MR, Winkelmann RK. Clinical and pathologic correlations in 96 patients with panniculitis, including 15 patients with deficient levels of alpha 1-antitrypsin . J Am Acad Dermatol. 1989;21:1192-1196.Crossref 14. Hodges J, Milward-Sadler G, Barbatis C, Wright R. Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis . N Engl J Med. 1981;304:557-560.Crossref 15. Du Bois R, Bernaudin J, Paakko P, et al. Human neutrophils express the alpha-1-antitrypsin gene and produce alpha-1-antitrypsin . Blood. 1991;77:2724-2730. 16. Kunitomo M, Jay M. Elastin fragment-induced monocyte chemotaxis: the role of desmosines . Inflammation. 1985;9:183-188.Crossref 17. Hunninghake G, Davidson J, Rennard S, et al. Elastin fragments attract macrophage precursors to diseased sites in pulmonary emphysema . Science . 1981;66:925-927.Crossref 18. Lee D, Cho B, Hervey J, Daynes R. The role of neutrophils in tissue localized cell-mediated immunologic responses , I. Region Immunol. 1988;1:149-162. 19. Hendrick SJ, Silverman AK, Solomon AR. α1-Antitrypsin deficiency associated with panniculitis . J Am Acad Dermatol. 1988;18:684-692.Crossref 20. Geller JD, Su WPD. A subtle clue to the histopathologic diagnosis of early α1-antitrypsin deficiency panniculitis . J Am Acad Dermatol. 1994;31:241-245.Crossref 21. Fornieri C, Quaglino D, Lungarella G, et al. Elastin production and degradation in cutis laxa acquisita . J Invest Dermatol. 1994;103:583-588.Crossref 22. Verhagen A, Woerdeman M. Post-inflammatory elastolysis and cutis laxa . Br J Dermatol. 1975;92:183-190.Crossref 23. Kibbi A, Zaynoun S, Kurban A, Najjar S. Acute febrile neutrophilic dermatosis (Sweet's syndrome): case report and review of the literature . Pediatr Dermatol. 1985;3:40-44.Crossref 24. Fisher B, Page E, Hanna W. Acral localized acquired cutis laxa . J Am Acad Dermatol. 1989;21:33-40.Crossref 25. Muster A, Bharati S, Herman J, et al. Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis . J Pediatr. 1983;102:243-248.Crossref 26. Levin D, Esterly N, Herman J, Boxall L. The Sweet syndrome in children . J Pediatr. 1981;99:73-78.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Sweet's Syndrome Leading to Acquired Cutis Laxa (Marshall's Syndrome) in an Infant With a1-Antitrypsin Deficiency

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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1995.01690220081015
Publisher site
See Article on Publisher Site

Abstract

Abstract Background: Marshall's syndrome is a rare pediatric skin disease that is characterized by acquired, localized neutrophilic dermatitis (Sweet's disease), followed by loss of elastic tissue in the dermis and cutis laxa. The cause of this syndrome is unknown. α1-Antitrypsin (α1-AT) deficiency is a codominantly inherited disorder of α1-AT, the major serum antiprotease active against a number of serine-type proteases. Observations: The first patient with classic Marshall's syndrome who had coexisting α1-AT deficiency and a review of other cases of Marshall's syndrome are pre- sented, and pathogenic mechanisms are discussed. Conclusions: A deficiency of α1-AT may allow proteases such as neutrophil elastase to destroy dermal elastin and, thus, produce cutis laxa in Marshall's syndrome. Other cases of acquired cutis laxa should be screened for α1-AT deficiency to further evaluate this association and to enable patients and their families to be counseled about possible systemic complications of α1-AT deficiency.(Arch Dermatol. 1995;131:1175-1177) References 1. Lewis F, Lewis-Jones S, Gipson M. Acquired cutis laxa with dermatitis herpetiformis and sarcoidosis . J Am Acad Dermatol. 1993;29:846-848.Crossref 2. Kerl H, Burg G, Hashimoto K. Fatal penicillin induced generalised post-inflammatory elastolysis (cutis laxa) . Am J Dermatopathol. 1983;3:267-276.Crossref 3. Marshall J, Heyl T, Weber HW. Postinflammatory elastolysis and cutis laxa . S Afr Med J. 1966;40:1016-1022. 4. Sweet R. An acute febrile neutrophilic dermatosis . Br J Dermatol. 1964;76: 349-356.Crossref 5. Koch S, Williams M. Acquired cutis laxa . Pediatr Dermatol. 1985;2:282-288.Crossref 6. Kemmett D, Hunter J. Sweet's syndrome . J Am Acad Dermatol. 1990;23:503-507.Crossref 7. Petrozzi J, Wartham T. Sweet's syndrome: unique local response to streptococcal antigen . Cutis. 1971;84:393-399. 8. Saxe N, Gordon W. Acute febrile neutrophilic dermatosis (Sweet's syndrome): four case reports . S Afr Med J. 1978;52:253-256. 9. Reed W, Horowitz R, Beighton P. Acquired cutis laxa: primary generalized elastolysis . Arch Dermatol. 1971;103:661-669.Crossref 10. Goltz R, Hult A, Goldfarb M, et al. Cutis laxa: a manifestation of generalized elastolysis . Arch Dermatol. 1965;92:373-387.Crossref 11. Wulfsberg EA, Hoffman DE, Cohen MM. Alpha-1-antitrypsin deficiency: impact of genetic discovery on medicine and society . JAMA. 1994;271:217-222.Crossref 12. Perlmutter D. The cellular basis for liver injury in alpha-1-antitrypsin deficiency . Hepatology. 1991;13:172-185.Crossref 13. Smith KC, Su WP, Pittelkow MR, Winkelmann RK. Clinical and pathologic correlations in 96 patients with panniculitis, including 15 patients with deficient levels of alpha 1-antitrypsin . J Am Acad Dermatol. 1989;21:1192-1196.Crossref 14. Hodges J, Milward-Sadler G, Barbatis C, Wright R. Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis . N Engl J Med. 1981;304:557-560.Crossref 15. Du Bois R, Bernaudin J, Paakko P, et al. Human neutrophils express the alpha-1-antitrypsin gene and produce alpha-1-antitrypsin . Blood. 1991;77:2724-2730. 16. Kunitomo M, Jay M. Elastin fragment-induced monocyte chemotaxis: the role of desmosines . Inflammation. 1985;9:183-188.Crossref 17. Hunninghake G, Davidson J, Rennard S, et al. Elastin fragments attract macrophage precursors to diseased sites in pulmonary emphysema . Science . 1981;66:925-927.Crossref 18. Lee D, Cho B, Hervey J, Daynes R. The role of neutrophils in tissue localized cell-mediated immunologic responses , I. Region Immunol. 1988;1:149-162. 19. Hendrick SJ, Silverman AK, Solomon AR. α1-Antitrypsin deficiency associated with panniculitis . J Am Acad Dermatol. 1988;18:684-692.Crossref 20. Geller JD, Su WPD. A subtle clue to the histopathologic diagnosis of early α1-antitrypsin deficiency panniculitis . J Am Acad Dermatol. 1994;31:241-245.Crossref 21. Fornieri C, Quaglino D, Lungarella G, et al. Elastin production and degradation in cutis laxa acquisita . J Invest Dermatol. 1994;103:583-588.Crossref 22. Verhagen A, Woerdeman M. Post-inflammatory elastolysis and cutis laxa . Br J Dermatol. 1975;92:183-190.Crossref 23. Kibbi A, Zaynoun S, Kurban A, Najjar S. Acute febrile neutrophilic dermatosis (Sweet's syndrome): case report and review of the literature . Pediatr Dermatol. 1985;3:40-44.Crossref 24. Fisher B, Page E, Hanna W. Acral localized acquired cutis laxa . J Am Acad Dermatol. 1989;21:33-40.Crossref 25. Muster A, Bharati S, Herman J, et al. Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis . J Pediatr. 1983;102:243-248.Crossref 26. Levin D, Esterly N, Herman J, Boxall L. The Sweet syndrome in children . J Pediatr. 1981;99:73-78.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Oct 1, 1995

References