An elastic tissue defect in dermatofibrosis lenticularis disseminata. Buschke-Ollendorff syndrome

Abstract

A patient with [the autosomal dominant condition] dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome) had a distinctive abnormality of the cutaneous elastic tissue. This abnormality, studied by histochemical and ultrastructural techniques, was characterized by the presence of hyalinized, thick fibers that entrapped normal bundles of collagen. Histochemically, these fibers stained like elastin but showed certain tinctorial characteristics of pre-elastin. Ultrastructurally, this elastin-like substance was composed of large clumps of electron-dense material with a fine fibrillar coating. The changes were sufficiently distinctive to be diagnosed by light microscopy.