Connective tissue nevusD23.l

Congenital, circumscribed malformation of the corial connective tissue with excessive formation of collagen and usually also elastic fibres. A distinction is made between the clinical and histological appearance:

• elastic nevus
• Connective tissue nevus, grossly nodular-disseminated
• Connective tissue nevus, lumbosacral
• Elastoma juvenile.

These diagnoses represent variants of one entity and may occur simultaneously in the same patient. Connective tissue nevi can occur in isolation or as part of hereditary syndromes, e.g. Pringle-Bourneville phacomatosis and Buschke-Ollendorf syndrome.

From birth, rarely occurs in childhood.

Pinhead to coin-sized, whitish to yellowish or skin-coloured, moderately coarse, flat oval to polygonal papules confluent to cobblestone-like foci. Often arranged in the cleft lines of the skin.

S.u. nevus elasticus; connective tissue nevus, grossly nodular-disseminated; connective tissue nevus, lumbosacral; elastoma juvenile.