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November 1986

Congenital Cutis Laxa Syndrome: Relation of Joint Dislocations to Oligohydramnios

Author Affiliations

Regional Genetic Center Department of Pediatrics; Department of Pediatrics State University of New York-Health Science Center at Syracuse 750 E Adams St Syracuse, NY 13210

Arch Dermatol. 1986;122(11):1241-1243. doi:10.1001/archderm.1986.01660230031005

To the Editor.—  Cutis laxa is a rare condition in which the skin hangs in loose folds, suggesting clothing too large for its wearer. All of the classic mendelian patterns of inheritance have been described in different kindreds. By their teens, affected children may appear older than their parents. This letter describes the clinical course and laboratory evaluation of a male infant with congenital cutis laxa, probably of the autosomal recessive type. This patient also exhibited congenital dislocation of the hips (as well as shoulders and radial heads) and general laxity of the joints. A curious finding was oligohydramnios detected by sonography, which may have begun as early as 15 weeks' gestation.

Report of a Case.—  The patient was born at 351/2 weeks' gestation to a 25-year-old woman (gravida 2, para 1, abortus 0) and her 28-year-old husband. The pregnancy was complicated by an appendectomy at seven weeks and by

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