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May 1956

The Ehlers-Danlos Syndrome: A Review of the Literature and Report of a Case with a Subgaleal Hematoma and Bell's Palsy

Author Affiliations

Chapel Hill, N. C.
From the Department of Pediatrics, University of North Carolina School of Medicine, and the Pediatric Service, North Carolina Memorial Hospital; Trainee, National Institute of Arthritis and Metabolic Diseases.

AMA Am J Dis Child. 1956;91(5):419-428. doi:10.1001/archpedi.1956.02060020421001

Edvard Ehlers (1863-1937), a German dermatologist, in 1901 was the first to describe a syndrome which included laxity of the skin and joints associated with a tendency to hemorrhage.1 In 1907, Paul Cohn recorded a similar syndrome with circumscribed changes in the skin.2 Henri Alexandre Danlos (1844-1912), a French physician, in 1908 described a syndrome of laxity of the skin with tumors about the elbows and knees resulting from chronic contusions.3 This case had been reported earlier by Hallopeau as a doubtful form of diabetic xanthomatosis.4 Since 1908 the symptoms comprising the Ehlers-Danlos syndrome have been characterized by the following features: (1) extreme laxity of the skin (dermatochalasis); (2) extreme laxity of the joints (arthrochalasia); (3) fragility of the skin (dermatorrhexis) and blood vessels. L. M. Pautrier first reported the pathologic findings in the case described by Danlos in 1908.5 The first American case recorded