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September 6, 1965

Avascular Necrosis of the Capital Femoral Epiphysis and Poikiloderma CongenitaleTwo Cases in Siblings

Author Affiliations

From the Department of Orthopedic Surgery, Ochsner Clinic, New Orleans.

JAMA. 1965;193(10):852-853. doi:10.1001/jama.1965.03090100098043

EPIPHYSEAL OSTEOCHONDROSIS associated with poikiloderma congenitale (Thomson's disease) has not been previously reported, to my knowledge, although a wide variety of other skeletal defects have been observed in association with this syndrome. In the cases reported here, such an association occurred in siblings.

Report of Cases 

CASE 1.—  A Puerto Rican boy, age 10 1/2 years, who had Legg-Calvé-Perthes disease on both sides of the body came to the US Naval Hospital in Portsmouth, Va, on Feb 21, 1965, for a follow-up examination. His prenatal development, birth, neonatal development, and early infancy were normal. At the age of one year, his hands, ankles, and feet became red and swollen. Later, the extensor surfaces of the knees and elbows and of the processes of the lumbar spine were similarly affected but not the skin of the face. The erythema and edema gradually disappeared, with resultant linear and punctate areas of cutaneous

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