We observed an overexpression of TLR-3 mRNA in patients with fibrotic lung disorders (IPF and CTD-IP) versus patients with nonfibrotic sarcoidosis, suggesting that different activity of this receptor could be one of the mechanisms able to explain the skew toward the Th2 cytokine pattern which characterizes the development of fibrosis. Here, TLR3 is linked to incontinentia pigmenti.