The Danlos-Ehlers syndrome is also called cutis hyperelastica and dermatorrhexis. Persons with this disorder constitute an undetermined percentage of professional contortionists and exhibitionists; they are spoken of as "india rubber men."
Ehlers1 was the first to describe a case, and Danlos2 established the syndrome. The condition is considered very infrequent. Prior to December 1939 only about 60 cases could be culled from the literature.3 There are four primary signs: (1) hyperelasticity of the skin, (2) hypermotility of the joints, (3) a tendency to form abnormal and deficient scar, and (4) small tumors of the skin. Coe and Silvers4 recently reviewed the literature and included as minor symptoms chilblains and acrocyanosis. The cause of the condition is unknown. It is classified in the group of congenital dystrophic anomalies.3 Biopsy of the skin reveals an increase in the elastic elements.5 Three families have been described in
Sullivan JD. THE DANLOS-EHLERS SYNDROME: Report of a Case with Transient Paralysis of the Vocal Cord. Arch NeurPsych. 1942;47(2):316–318. doi:https://doi.org/10.1001/archneurpsyc.1942.02290020132012
Customize your JAMA Network experience by selecting one or more topics from the list below.