Elastoderma — Disease of Elastin Accumulation within the Skin

Abstract

ELASTIC fibers, which consist of two distinct structures — elastin and the microfibrillar component — are responsible for the elasticity of tissues, such as the skin.1 2 3 4 The understanding of the normal biology of elastic fibers has been aided by the demonstration of elastin abnormalities in diseases.4 In this report we describe a disorder of elastic fibers that causes an excessive accumulation of elastin within the skin. The accumulation of elastin was documented by histopathological staining with elastic-tissue stains and by a fourfold increase in the desmosine concentration, as determined by a radioimmunoassay. However, electron microscopy of the elastic fibers revealed . . .