Onychomatricoma

Abstract

Onychomatricoma was first described in 1992 by Baran and Kint. Twenty cases have been reported to date, all of which are from Europe. This tumor has characteristic clinical, light microscopic, and ultrastructural features which have recently been refined andjustify its categorization as a novel tumor of the nail matrix. We examined a specimen sent in consultation by a large reference laboratory; despite improper orientation and absence of the involved nail plate, it had microscopic characteristics consistent with onychomatricoma. Soon thereafter, a second patient presented with a tumor arising in the nailbed that bore a superficial resemblance to onychomatricoma but showed the microscopic features of fibrokeratoma. We discuss the differentiation between these two lesions and point out that a distinction can be made even when incomplete material is submitted for review. To our knowledge, this is the first example of onychomatricoma to be reported from North America.