Cellular infiltrates in scleroderma skin

Abstract

The purpose of this study was to determine the frequency, distribution, and nature of cellular infiltrates in 108 skin biopsies from patients with systemic scleroderma (SS) and localized scleroderma (LS). Cellular infiltrates, perivascular or diffuse, were noted in 49% of SS and 84% of LS patients and consisted of lymphocytes, plasma cells, and macrophages. No correlation was noted between the presence or severity of skin cellular infiltrates and serum serologic abnormalities.Systemic scleroderma is a connective tissue disease that affects various organ systems, but particularly the skin, lungs, gastrointestinal tract, and kidneys. Localized scleroderma affects only the skin, and it is not presently known whether it represents a separate entity or whether it is a clinical variant of systemic scleroderma. The pathogenesis of scleroderma remains unknown, although current research implicates three pos-

A macrophage marker, siglec‐1, is increased on circulating monocytes in patients with systemic sclerosis and induced by type i interferons and toll‐like receptor agonists

“…In early phases of SSc, there are often perivascular and/or diffuse mononuclear infiltrates that include both T cells and macrophages (27). Our observations are consistent with the findings of Fleischmajer et al in that the Siglec-1ϩ macrophages are both perivascular and located between collagen bundles in the dermis (49). Thus, Siglec-1ϩ macrophages might play an important role as effector cells in fibrosis through cytokine release.…”

Altered blood B lymphocyte homeostasis in systemic sclerosis: Expanded naive B cells and diminished but activated memory B cells

“…SSc patients have autoantibodies that react to various intracellular components, such as DNA topoisomerase I, centromere, RNA polymerases, U1RNP, and U3RNP (2). In addition, hypergammaglobulinemia and polyclonal B cell hyperactivity are detected in SSc patients (3,4). Furthermore, a recent study demonstrated that down-regulation of B cell function leads to improved skin fibrosis in the tight-skin mouse, a genetic model of SSc (5).…”

Effector CD8+ T cells in systemic sclerosis patients produce abnormally high levels of interleukin‐13 associated with increased skin fibrosis

“…Several lines of evidence suggest that T cells are important in the pathogenesis of SSc, including observations that ␣/␤ and ␥/␦ T lymphocytes exhibit increased expression of activation markers (3,4), show signs of antigen-driven expansion (5), and dominate the inflammatory infiltrates in the skin and involved tissues during the early stages of the disease (6,7). T cells have been found to be necessary for the production of antitopoisomerase I antibodies in SSc (8).…”