Document Detail


Granulomatous dermatitis with pseudoxanthoma elasticum-like changes: report of a case in a patient with cystic fibrosis.
MedLine Citation:
PMID:  19917959     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: There is scant literature that documents pseudoxanthoma elasticum (PXE)-like histologic changes in the setting of inflammatory skin diseases. This article documents granulomatous dermatitis with PXE-like changes in a patient with cystic fibrosis. This is the first report of its kind, to our knowledge. OBSERVATIONS: A 33-year-old woman with cystic fibrosis developed a papular eruption on the flexural surfaces of the upper and lower extremities, which was initially treated with prednisone. A punch biopsy showed granulomatous inflammation and associated PXE-like changes. The combined histologic and clinical findings were most consistent with granuloma annulare. There was no family history of PXE or clinical manifestations of PXE. The rash gradually resolved itself over the next several months. CONCLUSIONS: There are few publications that document PXE-like changes in association with various inflammatory skin conditions. Thus, the clinical significance of this finding remains uncertain. This case and previous reports are discussed in the context of current molecular and genetic knowledge. It is hoped that greater awareness of this phenomenon will promote further investigation and elucidation of the clinical and biologic significance of PXE-like changes observed in biopsies of inflammatory skin disorders.
Authors:
Jeffrey B Tiger; Jill McKenzie; Don T Tran; John E Olerud; Evan George
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of dermatology     Volume:  145     ISSN:  1538-3652     ISO Abbreviation:  Arch Dermatol     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-11-17     Completed Date:  2009-12-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372433     Medline TA:  Arch Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1292-5     Citation Subset:  AIM; IM    
Affiliation:
School of Medicine, University of Washington, Medical Center, Seattle, WA, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Biopsy, Needle
Cystic Fibrosis / complications,  diagnosis*,  genetics
Dermatitis / complications,  genetics,  pathology*
Female
Follow-Up Studies
Gene Expression Regulation
Genetic Predisposition to Disease
Granuloma Annulare / complications,  genetics,  pathology*
Humans
Immunohistochemistry
Lower Extremity
Pseudoxanthoma Elasticum / complications,  genetics,  pathology*
Severity of Illness Index

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


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