Abstract

Caffey's disease is an inflammatory skeletal disorder of infancy manifested clinically by fever, soft-tissue swelling, and constitutional signs with radiographic evidence of periosteal new bone formation. Although prevalent between 1940 and 1960, nonfamilial cases have become extraordinarily rare. The authors report the sporadic occurrence of congenital Caffey's disease in a premature infant and note an interesting association with maternal herpes zoster early during gestation. The etiology of this mysterious disease is likely to remain elusive as new cases become scarce.

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