Grouped papules in Hurler-Scheie syndrome - 11/09/11
Abstract |
In a patient with Hurler-Scheie syndrome, a type of mucopolysaccharidosis (I H/S), an initial presentation was grouped papules on the extensor surfaces on the upper portions of the arms and legs. Other physical findings included progressive flexion contractures and mild developmental delay. The patient had deficient alpha-l-iduronidase activity, and electron microscopy showed large cytoplasmic vacuoles and lysosomes, consistent with Hurler-Scheie syndrome. Findings of grouped papules have not been previously reported in patients with this syndrome.
Le texte complet de cet article est disponible en PDF. This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation. |
Vol 35 - N° 5P2
P. 868-870 - novembre 1996 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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