Document Detail


Scleromyxedema myopathy: case report and review of the literature.
MedLine Citation:
PMID:  3056423     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a patient who had scleromyxedema associated with proximal myopathy. The histopathologic and electron microscopic features are presented. In addition, we review the clinical and pathologic features of all 9 previously reported patients with documented scleromyxedema and myopathy. Proximal (pharyngeal) or distal dysphagia (7 of 7 patients), elevated creatine kinase (5 of 8), elevated aldolase (3 of 3), and electromyographically demonstrated myopathy (7 of 7) were usual features. Four patients had muscle biopsies that showed myofibril vacuolar changes, but inflammation was infrequent (2 cases). Our patient responded to oral prednisone and weekly intravenous methotrexate with improvement of the erythroderma, papular rash, and muscle strength.
Authors:
D J Helfrich; E R Walker; A J Martinez; T A Medsger
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  31     ISSN:  0004-3591     ISO Abbreviation:  Arthritis Rheum.     Publication Date:  1988 Nov 
Date Detail:
Created Date:  1988-12-16     Completed Date:  1988-12-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1437-41     Citation Subset:  AIM; IM    
Affiliation:
Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania.
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MeSH Terms
Descriptor/Qualifier:
Arm
Biopsy
Deglutition Disorders / complications
Electromyography
Face
Female
Humans
Middle Aged
Muscles / pathology,  ultrastructure
Muscular Diseases / complications*,  pathology,  physiopathology
Skin Diseases / complications*,  pathology,  physiopathology

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


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