July 1987

Picture of the Month

Author Affiliations

(Contributor); (Section Editor)
Contributed from the Alberta Children's Hospital, University of Calgary.

Am J Dis Child. 1987;141(7):803-804. doi:10.1001/archpedi.1987.04460070105036

The findings listed below are best associated with which of the above figures:

  • Mitral valve prolapse.

  • Frequently present in obese and dark complexioned individuals or patients with certain endocrine disorders.

  • Neuroblastoma.

Denouement and Discussion  Fig 1.—Hyperextensibility of joints in patient with Ehlers-Danlos syndrome.Fig 2.—Horner's syndrome (miosis, ptosis, and heterochromia iridis).Fig 3.—Acanthosis nigricans.(a) Hyperextensibility of joints (Fig 1) and hyperelasticity of skin are cardinal features of Ehlers-Danlos syndrome. In this syndrome, patients may have mitral valve prolapse with a midsystolic click and late systolic murmur. A defect in connective tissue, most likely in collagen cross-linking, is present. The skin is very fragile, resulting in ecchymoses with minor trauma, bleeding, poor healing, and atrophic scar formation. There are numerous types of Ehlers-Danlos syndrome, and their manifestations and inheritance patterns vary.(b) Horner's syndrome results from obstruction or interruption of the sympathetic outflow from the hypothalamus to

First Page Preview View Large
First page PDF preview
First page PDF preview