Systemic sclerosis: a prototypic multisystem fibrotic disorder
J. Clin. Invest. John Varga, et al. 117:557 doi:10.1172/JCI31139 [Go to this article.]

Figure 2
Skin inflammation and fibrosis in SSc. (A) In early diffuse cutaneous SSc, moderate fibrosis in the upper dermis and at the dermal-epidermal junction is accompanied by evidence of keratinocyte hypertrophy with a flattening of the epidermis, leading to loss of reticular structure and decreased length of rete pegs (fingerlike structures that project up from the dermis and down from the epidermis, increasing the area of contact between the layers of the skin). In addition, inflammatory infiltrates are found in the dermis and near the dermal-epidermal junction, predominantly around small blood vessels. (B) Early-stage diffuse disease showing profound dermal inflammation characterized by perivascular mononuclear cellular infiltrate composed of monocytes and activated lymphocytes, with perivascular fibrosis and loss of pericytes and vessel integrity. (C) In established fibrosis, collagen accumulation leads to dermal thickening and the deposition of dense and closely packed collagen fibers throughout the dermis, with the loss of the microvasculature and dermal structures and the dermis-subcutaneous adipose tissue interface. All images are stained with H&E and photographed using a Zeiss Axioscope confocal microscope under light field. Original magnification, ×100 (A and C); ×200 (B).