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June 1966


Arch Dermatol. 1966;93(6):772-775. doi:10.1001/archderm.1966.01600240138028

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Sjögren-Larsson Syndrome (in a Kinship of Three Siblings). Presented by Dr. Victor Selmanowitz. 

Case 1.—  A 13-year-old boy of Italian extraction who has long been known to have congenital ichthyosiform erythroderma and cerebral palsy, was admitted to University Hospital eight months ago for surgical correction of "tight heel cords" and "equinus deformities" of both feet secondary to spastic diplegia.Both parents, who are normal by history and on physical examination, come from the small community of San Leucio, a suburb of Benvenuto, Italy. There is no parental consanguinity. Two siblings show features of the Sjögren-Larsson syndrome while a brother and three half-siblings from the same father are free of involvement.The mother's pregnancy was full term and uneventful. Labor was prolonged but no fetal distress was noted. At birth the patient had diffuse redness of the skin which gradually faded during childhood when the skin became dry,

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