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Chediak-Higashi Syndrome With Severe Cutaneous Involvement: Occurrence in Two Brothers 14 and 15 Years of Age

Chediak-Higashi Syndrome With Severe Cutaneous Involvement: Occurrence in Two Brothers 14 and 15... Abstract THE Chediak-Higashi syndrome is a rare hereditary disorder. It is transmitted as an autosomal recessive trait and only 34 cases have been reported previously. The literature concerning this disorder has been reviewed extensively by Kritzler et al.1 In the present report, the occurrence of this syndrome is described in two brothers who are the fifth and sixth cases known to have survived past the age of 10 years and who exhibit severe cutaneous manifestations. While the cutaneous findings will be stressed, hematologic, ocular, neural, and visceral abnormalities will be discussed as they pertain to these patients. Report of Cases Case 1. —A 15-year-old white boy was admitted to the University of Virginia Hospital on Aug 14, 1965, because of extensive, severe cutaneous ulcers. From 3 months of age he had developed recurrent crops of cutaneous abscesses of the trunk and legs which started as small pustules and enlarged rapidly References 1. Kritzler, R.A., et al: Chediak-Higashi Syndrome: Cytologic and Serum Lipid Observations in a Case and Family , Amer J Med 36:583-594, 1964.Crossref 2. Saraiva, L.G., et al: Anomalous Panleukocytic Granulation , Blood 14:1112-1127, 1959. 3. Bernard, J., et al: Un cas de maladie de chediak-steinbrink-higashi: Etude clinique et cytologique , Presse Med 68:563-566, 1960. 4. White, J.G.: The Chediak-Higashi Syndrome: A Possible Lysosomal Disease , Blood , to be published. 5. Stegmaier, O.C., and Schneider, L.A.: Chediak-Higashi Syndrome: Dermatologic Manifestations , Arch Derm 91:1-9, 1965.Crossref 6. Pierini, D.O., and Abulafia, J.: Manifestaciones Cutaneas Del Sindrome de Chediak-Higashi , Arch Argent Derm 8:23-31, 1958 7. Year Book of Dermatology , 1958-1959, p 291. 8. Windhorst, D.B.; Zelickson, A.S.; and Good, R. A.: Chediak-Higashi Syndrome: Hereditary Gigantism of Cytoplasmic Organelles , Science 151:81-83, 1966.Crossref 9. Gilloon, J.R.; Pease, G.L.; and Mills, S.D.: Chediak-Higashi Anomaly of the Leukocytes: Report of Case , Proc Staff Meet Mayo Clin 35:635-640, 1960. 10. Donohue, W.L., and Bain, H.W.: Chediak-Higashi Syndrome: A Lethal Familial Disease With Anomalous Inclusions in the Leukocytes and Constitutional Stigmata: Report of a Case With Necropsy , Pediatrics 20:416-430, 1957. 11. Spencer, W.H., and Hogan, M.J.: Ocular Manifestations of Chediak-Higashi Syndrome. Report of a Case With Histopathologic Examination of Ocular Tissues , Amer J Ophthal 50:1197-1203, 1960. 12. Johnson, D.L., et al: Histopathology of Eyes in Chediak-Higashi Syndrome , Arch Ophthal 75:84-88, 1966.Crossref 13. Myers, J.P., et al: Pathological Findings in the Central and Peripheral Nervous Systems in Chediak-Higashi's Disease: The Finding of Cytoplasmic Neuronal Inclusions , J Neuropath Exp Neurol 22:357, 1963. 14. Page, A.R., et al: The Chediak-Higashi Syndrome , Blood 20:330-343, 1962. 15. White, J.G.: Virus-like Particles in the Peripheral Blood Cells of Two Patients With Chediak-Higashi Syndrome , Cancer 19:877-884, 1966.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Chediak-Higashi Syndrome With Severe Cutaneous Involvement: Occurrence in Two Brothers 14 and 15 Years of Age

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1967.00290220131010
Publisher site
See Article on Publisher Site

Abstract

Abstract THE Chediak-Higashi syndrome is a rare hereditary disorder. It is transmitted as an autosomal recessive trait and only 34 cases have been reported previously. The literature concerning this disorder has been reviewed extensively by Kritzler et al.1 In the present report, the occurrence of this syndrome is described in two brothers who are the fifth and sixth cases known to have survived past the age of 10 years and who exhibit severe cutaneous manifestations. While the cutaneous findings will be stressed, hematologic, ocular, neural, and visceral abnormalities will be discussed as they pertain to these patients. Report of Cases Case 1. —A 15-year-old white boy was admitted to the University of Virginia Hospital on Aug 14, 1965, because of extensive, severe cutaneous ulcers. From 3 months of age he had developed recurrent crops of cutaneous abscesses of the trunk and legs which started as small pustules and enlarged rapidly References 1. Kritzler, R.A., et al: Chediak-Higashi Syndrome: Cytologic and Serum Lipid Observations in a Case and Family , Amer J Med 36:583-594, 1964.Crossref 2. Saraiva, L.G., et al: Anomalous Panleukocytic Granulation , Blood 14:1112-1127, 1959. 3. Bernard, J., et al: Un cas de maladie de chediak-steinbrink-higashi: Etude clinique et cytologique , Presse Med 68:563-566, 1960. 4. White, J.G.: The Chediak-Higashi Syndrome: A Possible Lysosomal Disease , Blood , to be published. 5. Stegmaier, O.C., and Schneider, L.A.: Chediak-Higashi Syndrome: Dermatologic Manifestations , Arch Derm 91:1-9, 1965.Crossref 6. Pierini, D.O., and Abulafia, J.: Manifestaciones Cutaneas Del Sindrome de Chediak-Higashi , Arch Argent Derm 8:23-31, 1958 7. Year Book of Dermatology , 1958-1959, p 291. 8. Windhorst, D.B.; Zelickson, A.S.; and Good, R. A.: Chediak-Higashi Syndrome: Hereditary Gigantism of Cytoplasmic Organelles , Science 151:81-83, 1966.Crossref 9. Gilloon, J.R.; Pease, G.L.; and Mills, S.D.: Chediak-Higashi Anomaly of the Leukocytes: Report of Case , Proc Staff Meet Mayo Clin 35:635-640, 1960. 10. Donohue, W.L., and Bain, H.W.: Chediak-Higashi Syndrome: A Lethal Familial Disease With Anomalous Inclusions in the Leukocytes and Constitutional Stigmata: Report of a Case With Necropsy , Pediatrics 20:416-430, 1957. 11. Spencer, W.H., and Hogan, M.J.: Ocular Manifestations of Chediak-Higashi Syndrome. Report of a Case With Histopathologic Examination of Ocular Tissues , Amer J Ophthal 50:1197-1203, 1960. 12. Johnson, D.L., et al: Histopathology of Eyes in Chediak-Higashi Syndrome , Arch Ophthal 75:84-88, 1966.Crossref 13. Myers, J.P., et al: Pathological Findings in the Central and Peripheral Nervous Systems in Chediak-Higashi's Disease: The Finding of Cytoplasmic Neuronal Inclusions , J Neuropath Exp Neurol 22:357, 1963. 14. Page, A.R., et al: The Chediak-Higashi Syndrome , Blood 20:330-343, 1962. 15. White, J.G.: Virus-like Particles in the Peripheral Blood Cells of Two Patients With Chediak-Higashi Syndrome , Cancer 19:877-884, 1966.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Apr 1, 1967

References