Document Detail


A case-control study of cutaneous signs in adult patients with Marfan disease: Diagnostic value of striae.
MedLine Citation:
PMID:  21112669     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
BACKGROUND: Marfan syndrome (MS) (OMIM 154700) has been associated with various skin manifestations.
OBJECTIVE: We sought to clarify the value of skin signs in patients with MS.
METHODS: This was a case-control study. A total of 61 consecutive patients (median age: 34 years) seen in the French Reference Centre for MS and Related Disorders and with a confirmed diagnosis of MS were paired with 61 age-, sex-, and height-paired control subjects. All had a structured interview and standardized dermatologic examination. The gold standard for MS diagnosis was the Ghent criteria.
RESULTS: Striae of any type were significantly (P = .0001) more frequent in patients with MS (92%) than in control subjects (61%), but specificity was low (39%, 95% confidence interval [CI] 27-52). Striae on unusual locations (other than buttock, hip, or thigh) were more frequent in patients with MS (66%) than in control subjects (16%) (P < .0001). This finding had a high specificity (84%, 95% CI 74-93), without notably decreasing sensitivity (66%, 95% CI 54-77). Hypertrophic, large, or atrophic surgical or posttraumatic, frequently hypopigmented or hyperpigmented, scars were present in 46% of patients with MS and 21% of control subjects (P = .007). Sensitivity was 46% (95% CI 34-58) and specificity 79% (95% CI 67-87). Atypical striae in some control subjects could be attributed to intensive practice of sports.
LIMITATION: A few control subjects were selected from patients consulting the MS center but without a diagnosis of MS.
CONCLUSION: Striae are a good diagnostic criterion for MS, particularly when arising in unusual sites. Other reported skin signs of MS are infrequent. (J Am Acad Dermatol doi:10.1016/j.jaad.2010.01.032.).
Authors:
Monica Ledoux; Alain Beauchet; Christophe Fermanian; Catherine Boileau; Guillaume Jondeau; Philippe Saiag
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Publication Detail:
Type:  Journal Article     Date:  2010-11-26
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  64     ISSN:  1097-6787     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-01-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  290-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.
Affiliation:
Department of Dermatology and Research Unit EA 4339 "Skin, Environment, Cancer," Université Versailles St-Quentin, Ambroise Paré Hospital, Boulogne, France.
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