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January 13, 1969

Fatal Varicella

Author Affiliations

Johns Hopkins Hospital Baltimore

JAMA. 1969;207(2):370. doi:10.1001/jama.1969.03150150082037

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To the Editor:—  In connection with the report of fatal varicella by Hook and his colleagues (206:305, 1968) it might be noted that this very rare cause of death is observed with significant frequency in persons with cartilage-hair hypoplasia (CCH). Cartilage-hair hypoplasia is a recessively inherited skeletal dysplasia which was first discovered in numerous cases among the Old Order Amish. However, it has since then been observed in non-Amish persons in many parts of the world (eg, Journal of Bone Joint Surg50A:1245, 1968).In our report on this condition (Bull Hopkins Hosp116:285, 1965), my colleagues and I noted that one child with CHH died at age 6 of chickenpox and another at age 9. Furthermore, at least three others had such virulent attacks that smallpox was seriously considered. Very severe chickenpox in non-Amish patients with CHH has been described to me. Our studies have not uncovered the