Connective Tissue Nevus

Abstract

The connective tissue nevus is a hamartoma of collagen tissue producing a dermal tumor which in confluent plaques gives the appearance of peau de chagrin. Cases have been reported, especially in the European literature, under a variety of names: nevus elasticus regionis mammariae,¹ pflastersteinförmiger Bindegewebesnaevus,² Bindegewebesnaevus,^3-5 dystrophie elastique folliculaire,⁶ naevus pseudocolloid perifolliculaire,⁷ juvenile elastoma,⁸ collagénome eruptif.^9-10 All of these reports and discussions differ in their interpretation of the histologic evidence, some emphasizing the collagenous change and others the changes in elastic tissue. A similar lesion seen associated with tuberous sclerosis has been termed shagreen skin (chagrin haut, peau de chagrin). Riehl¹¹ in 1935 defined connective tissue nevus as that lesion of chagrin haut seen in patients with adenoma sebaceum. Recently Nickel and Reed¹² pointed out the clinical and histologic similarities between connective tissue nevus and the fibrous dermal proliferations observed on