Clear cell syringoma(CCS), which is histological variant form of syringoma that is clinically indistinguishable from ordinary syringoma. It has large vacuolated cytoplasm (glycogen) with a small hyperchromatic nucleus in the pericytoplasm. Many reports have suggested that CCS was associated with diabetes mellitus. The case without history of diabetes mellitus has been rarely reported. A 50-year-old woman presented with a 1-year history of bilateral skin-colored to erythematous papules on her periocular area and perioral area. The papules had progressively grown in number and dimension. The patient did not have a diabetes mellitus and other systemic diseases. Histopathologically, nests of clear cells with tadpole appearance were distributed in the upper and middle layer of the dermis. Tumor cells were composed of two or more layers with pale clear cytoplasm and ductal structures. Clear cells showed positivity with periodic acid of Schiff stains, which was absent after digestion with diastase. It was consistent with CCS. Herein, we report a rare case of CCS localized on the periocular area and perioral area without diabetes mellitus.
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