Ultrastructural changes in disseminated connective tissue nevi

Skin biopsy specimens from a patient with disseminated connective tissue nevi and from her son and daughter who had no such clinical alterations were studied with the electron microscope. The nevi showed increased amounts of thick elastic fibres, which were composed of irregularly arranged, often finger-like, subunits surrounded by a thready material. Both structures probably represent malformed components of elastic fibres, the thready material representing malformed elastic fibrils. Many of the collagen fibrils showed increased diameters and twistings, which alterations are believed to represent malformation of collagen. The fibroblasts showed changes which might be related to a pathological protein synthesis. Examination of biopsy specimens from normal skin of the patient and from the skin of her son and daughter failed to demonstrate similar alterations. The present case is regarded as an entity, distinct from dermatofibrosis lenticularis disseminata and from shagreen patch.