We describe the case of a painful primary cutaneous leiomyosarcoma tha
t developed on the back of a 54-year-old white male over a 6-year peri
od. The lesion had been sampled by punch technique and had been origin
ally diagnosed as cutaneous sclerosis. Histologic examination of excis
ional tissue revealed a diffuse spindle cell neoplasm in the dermis th
at extended into the subcutis. There was extensive sclerosis and spars
e cellularity in the deep portion and in several zones throughout the
tumor. Immunostaining for desmin was negative, although stains for vim
entin and smooth muscle actin were both strongly positive. Sclerotic c
utaneous leiomyosarcoma should be recognized as a distinct but unusual
variant of leiomyosarcoma that may be difficult to diagnose because o
f extensive sclerosis. Lesions may be painful and should be considered
in the differential diagnosis of painful cutaneous neoplasms of the s
kin.