We report a case of a previously undescribed benign neoplasm character
ized by a poorly circumscribed proliferation of clear cells, arranged
both singly and in elongated fascicles oriented in haphazard fashion w
ithin the middle and lower reticular dermis, The fascicles consisted o
f spindle-shaped cells that were periodic acid-Schiff positive and dia
stase labile, These cells were characterized by oval, hyperchromatic,
cigar-shaped nuclei and clear cytoplasm. Immunohistochemically, neopla
stic cells were diffusely positive for smooth muscle actin (1A4), comm
on muscle actin (HHF-35), and vimentin, Immunoreactivity for fibronect
in was also focally noted. The neoplasm, which we designated clear-cel
l smooth muscle tumor of the skin, should be distinguished from smooth
muscle hamartoma and other smooth muscle proliferations at cutaneous
level, We highlight in this case report the histopathologic differenti
al diagnosis among other tumors with myofibroblastic, melanocytic, and
neural differentiation.