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Comment
. 2010 Mar 17;2(23):23ps13.
doi: 10.1126/scitranslmed.3000949.

Stiff and tight skin: a rear window into fibrosis without inflammation

Enrico V Avvedimento  1 , Armando Gabrielli
Affiliations
Comment

Stiff and tight skin: a rear window into fibrosis without inflammation

Enrico V Avvedimento et al. Sci Transl Med. .

Abstract

In this issue of Science Translational Medicine, a report by Loeys et al. on mutations in the fibrillin-1 gene in patients with skin fibrosis (stiff skin) adds a new piece of information on a connective tissue disorder that resembles systemic sclerosis, an autoimmune disease characterized by skin fibrosis and visceral organ involvement. Here, we discuss the implications of these findings, as well as new opportunities for targeted therapy for fibrosis.

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Comment on

  • Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.
    Loeys BL, Gerber EE, Riegert-Johnson D, Iqbal S, Whiteman P, McConnell V, Chillakuri CR, Macaya D, Coucke PJ, De Paepe A, Judge DP, Wigley F, Davis EC, Mardon HJ, Handford P, Keene DR, Sakai LY, Dietz HC. Loeys BL, et al. Sci Transl Med. 2010 Mar 17;2(23):23ra20. doi: 10.1126/scitranslmed.3000488. Sci Transl Med. 2010. PMID: 20375004 Free PMC article.

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