Abstract

Eccrine nevi are rare lesions, with less than 50 dedicated papers in the literature. These hamartomatous lesions often present in childhood and are associated with hyperhidrosis. In some circumstances, they may present as domed/polypoid lesions in the coccygeal area and may be clinically mistaken for skin tags or worrisome for spinal anomalies. Coccygeal polypoid eccrine nevi are exceedingly rare, with only six reported cases in the literature. The majority occur in young girls and are reportedly present at birth. We present the case of a 9-year-old female with a congenital polypoid lesion of her gluteal cleft, approximately 2 cm caudal to the apex of the crease, that was associated with a small sacral dimple. No associated sinus, mass or spinal malformation was appreciated. The lesion was assumed to be an acrochordon and was removed at the parents’ request, as it was bothersome to the patient. Grossly, it was a 0.4 x 0.2 x 0.2 cm excrescence of fleshy tissue protruding from a skin ellipse. Histologic examination revealed a polypoid lesion with a central core of numerous eccrine glands and convoluted eccrine ducts. Other adnexal structures were not identified within the lesion. The histologic findings are diagnostic of a coccygeal polypoid eccrine nevus. While this is a benign lesion, this case adds to the few cases in the literature and reminds both dermatopathologists and pediatric pathologists to include this extraordinary entity within the differential of a coccygeal skin tag.