A case-control study of cutaneous signs in adult patients with Marfan disease: Diagnostic value of striae - 10/08/11
Reprint requests: Philippe Saiag, MD, PhD, Department of Dermatology and EA 4339, Ambroise Paré Hospital, 9 Avenue Charles de Gaulle, 92104 Boulogne, France.Abstract |
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.)
Le texte complet de cet article est disponible en PDF.Key words : Marfan syndrome, skin manifestation, striae
Abbreviations used : CI, MS, TGF
Plan
| Dr Jondeau and French Reference Centre for Marfan Syndrome and Related Disorders moved to the Bichat Hospital, Paris, France, after completion of the study. |
|
| Funding sources: None. |
|
| Conflicts of interest: None declared. |
Vol 64 - N° 2
P. 290-295 - février 2011 Retour au numéro
Article précédent
- Skin cancer education and early detection at the beach: A randomized trial of dermatologist examination and biometric feedback
- Karen M. Emmons, Alan C. Geller, Elaine Puleo, Sanghamitra S. Savadatti, Stephanie W. Hu, Susan Gorham, Andrew E. Werchniak, Dana-Farber Skin Cancer Screening Group
- Calcium may preferentially deposit in areas of elastic tissue damage
- Rupa Pugashetti, Kanade Shinkai, Beth S. Ruben, Marc E. Grossman, Janet Maldonado, Lindy P. Fox
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?