Document Detail


Grouped papules in Hurler-Scheie syndrome.
MedLine Citation:
PMID:  8912609     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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-induronidase 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.
Authors:
J A Schiro; S B Mallory; L Demmer; S B Dowton; M C Luke
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  35     ISSN:  0190-9622     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  1996 Nov 
Date Detail:
Created Date:  1996-12-17     Completed Date:  1996-12-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  868-70     Citation Subset:  IM    
Affiliation:
Division of Dermatology, Washington University School of Medicine, St. Louis, Missouri, USA.
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Humans
Male
Mucopolysaccharidosis I / complications*
Skin Diseases, Papulosquamous / etiology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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