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April 1969, Vol 117, No. 4 >
Article | April 1969

Cutis Laxa FREE

Elizabeth Maxwell, MD; Nancy B. Esterly, MD
[+] Author Affiliations

Received for publication June 5, 1968.

Reprint requests to the Department of Pediatrics, The Johns Hopkins Hospital, Baltimore 21205 (Dr. Maxwell).


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

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

REFERENCES

1 +
Goltz, R.W., and Hult, A.M.:  Generalized Elastolysis (Cutis Laxa) and Ehlers-Danlos Syndrome (Cutis Hyperelastica) , South Med J 58: 848-854 ( (July) -Sept) 1965;.
2 +
Goltz, R.W., et al:  Cutis Laxa , Arch Derm 92:373-387 ( (Oct) ) 1965;.
3 +
Hayden, J.G.; Talner, N.S.; and Klaus, S.M.:  Cutis Laxa Associated With Pulmonary Artery Stenosis , J Pediat 72:506-509 ( (April) ) 1968;.
4 +
Talbot, F.B.:  Metabolism Study of a Case Simulating Premature Senility , Monatschr Kinderh 25:643-646 ( (March) ) 1923;.
5 +
McKusick, V.A.: Heritable Disorders of Connective Tissue , St. Louis: C. V. Mosby Co., 1966;, p 181.
6 +
Nitishin, A.:  Cutis Laxa With Bilateral Pulmonary Emphysema , Arch Derm 95:334-336 ( (March) ) 1967;.
7 +
Christiaens, L., et al:  Emphyseme Congenital et Cutis Laxa , Presse Med 62:1799-1801 ( (Dec 25) ) 1954;.
8 +
Bakker, B.J.:  Cutis Laxa Universalis and Lungenemphysem , Hautarzt 10:371-373 ( (Aug) ) 1959;.
9 +
Robinson, H.S., and Ellis, F.A.:  Cutis Laxa , Arch Derm 77:656-665 ( (June) ) 1958;.
10 +
Schneider, R.B., et al:  Serum Elastase Inhibitor Levels in Pregnancy and Under Endocrine Influence , J Appl Physiol 15:992-994 ( (Nov) ) 1960;.
11 +
Banga, I.:  Determination of Elastase Inhibitor by means of Orecein-Elastin , Acta Physiol Acad Sci Hung 24:1-9, 1963;.

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References

1 +
Goltz, R.W., and Hult, A.M.:  Generalized Elastolysis (Cutis Laxa) and Ehlers-Danlos Syndrome (Cutis Hyperelastica) , South Med J 58: 848-854 ( (July) -Sept) 1965;.
2 +
Goltz, R.W., et al:  Cutis Laxa , Arch Derm 92:373-387 ( (Oct) ) 1965;.
3 +
Hayden, J.G.; Talner, N.S.; and Klaus, S.M.:  Cutis Laxa Associated With Pulmonary Artery Stenosis , J Pediat 72:506-509 ( (April) ) 1968;.
4 +
Talbot, F.B.:  Metabolism Study of a Case Simulating Premature Senility , Monatschr Kinderh 25:643-646 ( (March) ) 1923;.
5 +
McKusick, V.A.: Heritable Disorders of Connective Tissue , St. Louis: C. V. Mosby Co., 1966;, p 181.
6 +
Nitishin, A.:  Cutis Laxa With Bilateral Pulmonary Emphysema , Arch Derm 95:334-336 ( (March) ) 1967;.
7 +
Christiaens, L., et al:  Emphyseme Congenital et Cutis Laxa , Presse Med 62:1799-1801 ( (Dec 25) ) 1954;.
8 +
Bakker, B.J.:  Cutis Laxa Universalis and Lungenemphysem , Hautarzt 10:371-373 ( (Aug) ) 1959;.
9 +
Robinson, H.S., and Ellis, F.A.:  Cutis Laxa , Arch Derm 77:656-665 ( (June) ) 1958;.
10 +
Schneider, R.B., et al:  Serum Elastase Inhibitor Levels in Pregnancy and Under Endocrine Influence , J Appl Physiol 15:992-994 ( (Nov) ) 1960;.
11 +
Banga, I.:  Determination of Elastase Inhibitor by means of Orecein-Elastin , Acta Physiol Acad Sci Hung 24:1-9, 1963;.

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