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April 1967

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

Author Affiliations

Charlottesville, Va

From the departments of dermatology (Dr. Weary) and internal medicine (Dr. Bender), University of Virginia School of Medicine, Charlottesville, Va.

Arch Intern Med. 1967;119(4):381-386. doi:10.1001/archinte.1967.00290220131010

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

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