Document Detail


Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.
MedLine Citation:
PMID:  18712025     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.
Authors:
Erika M Summers; Christina G Kendrick
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Cutis     Volume:  82     ISSN:  0011-4162     ISO Abbreviation:  Cutis     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-08-20     Completed Date:  2008-10-30     Revised Date:  2013-09-12    
Medline Journal Info:
Nlm Unique ID:  0006440     Medline TA:  Cutis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  55-9     Citation Subset:  IM    
Affiliation:
Department of Internal Medicine, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA. esummers@bidmc.harvard.edu
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MeSH Terms
Descriptor/Qualifier:
Amyloidosis / complications*,  pathology*,  therapy
CREST Syndrome / complications*,  pathology*,  therapy
Female
Humans
Middle Aged
Skin Diseases, Metabolic / complications*,  pathology*,  therapy

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