Extracellular microfibrils are increased in localized and systemic scleroderma skin.

Abstract

Extracellular microfibrils, about 10 nm thick with a hollow core have been found in most organs as free bundles or in association with elastic fibrils. Histochemistry of the dermis of 4 patients with localized and 6 with systemic scleroderma revealed numerous fine elastic fibrils in areas of fibrosis. Immunofluorescence and immunoelectron microscopy were performed with antibodies against fibrillin and amorphous elastin. The lower dermis revealed an increase in 10-nm microfibrils interspersed between collagen fibrils. These microfibrils stained for fibrillin but not for amorphous elastin. Fibrosis in localized and systemic scleroderma involves the deposition of collagen fibrils and microfibrils.