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April 1969

Cutis Laxa

Author Affiliations

From the Department of Pediatrics, The Johns Hopkins Hospital, Baltimore. Doctor Esterly is now with La Rabida-University of Chicago Institute, Chicago.

Am J Dis Child. 1969;117(4):479-482. doi:10.1001/archpedi.1969.02100030481018

CUTIS LAXA is a rare, congenital skin disease with associated internal manifestations, primarily pulmonary emphysema, which limit the life span of the patient. It is to be distinguished from Ehlers-Danlos syndrome (cutis hyperelastica), also a multisystem disorder. The basic abnormality is not clearly defined but is probably a structural defect of the elastic tissue in several organ systems.

Report of a Case  A 2-year-old Negro boy (JHH-1203951) was first seen at The Johns Hopkins Hospital for evaluation of recurrent bronchiolitis and lax skin. He was the only child of nonrelated Negro parents. Pregnancy and delivery were normal; peculiar facies was the only abnormal physical finding.The patient was well until 5 months of age when wheezing and tachypnea were first observed. Thereafter he was treated frequently in the outpatient department for bronchiolitis and asthma but was relatively unresponsive to the usual therapeutic measures.At age 2, he was in the

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