Clear cell acanthoma

Summary Clear cell acanthoma is a rarely diagnosed tumor with variable clinical morphology that is usually only recognized by its histopathological features. The primary lesion is a red papule a few millimeters in diameter that often occurs as a single lesion on the lower extremities. In dermoscopy, resemblance of the vessels to a string of pearls is a largely specific finding of clear cell acanthoma. In contrast to the initially uncharacteristic clinical findings, histopathology of clear cell acanthomas is characterized by a typical compact, well‐demarcated acanthosis consisting of pale‐staining, PAS‐reactive keratinocytes. As etiology and pathogenesis are both unclear, nosology of clear cell acanthoma is also controversial, with an ongoing debate as to its classification as cutaneous neoplasia or reactive inflammatory dermatosis.