Nevoid anomalies of the connective tis[ill] the skin are uncommon. Prakken,1 in [ill] uncovered 48 cases in the literature, but his compilation was not entirely complete. Described and named are various clinical forms, including juvenile elastosis, sclerodermoid nevus, zosteriform nevus, and cobblestone nevus, in which abnormalities of collagen or elastic fibers or both are concerned. "Collagenous plaques," or "shagreen patches," are connective tissue nevi frequently associated with adenoma sebaceum.2,3 So long as nothing is known concerning the etiology or pathogenesis of connective tissue nevi, no useful purpose is apt to be served by separating the various morphologic manifestations into rigid taxonomic pigeonholes. The term connective tissue nevus ("nevus" signifying a congenital deviation in structure, synonymous with hamartoma) is a suitable one for encompassing all the clinical and histopathologic variants.
Prakken1 synthesized the following clinical description from his survey of reported cases:
The smooth, white, or yellow, flat, slightly
SMITH AD, WAISMAN M. Connective Tissue Nevi: Familial Occurrence and Association with Osteopoikilosis. AMA Arch Derm. 1960;81(2):249–252. doi:10.1001/archderm.1960.03730020085013
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