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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

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