A case-control study of cutaneous signs in adult patients with Marfan disease: Diagnostic value of striae

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• 作者：Monica Ledoux MD^a ; Alain Beauchet MD^b ; Christophe Fermanian PhD^b ; Catherine Boileau MD ; PhD^c ; Guillaume Jondeau MD ; PhD^c ; Philippe Saiag MD ; PhD^a ; ^{philippe.saiag@apr.aphp.fr}
• 关键词：Marfan syndrome ; skin manifestation ; striae
• 刊名：Journal of the American Academy of Dermatology
• 出版年：2011
• 出版时间：February 2011
• 年：2011
• 卷：64
• 期：2
• 页码：290-295
• 全文大小：164 K

文摘

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.)