Sclerosing sweat duct carcinoma of the eyelid margin: unusual presentation of a rare tumor

OBJECTIVE: Sclerosing sweat duct carcinoma (SSDC) is a rare, slow-growing, locally invasive skin tumor of eccrine and pilar origin. It is usually located on the face, particularly the upper lip, cheek, and forehead. It has been infrequently reported on the eyelid, secondarily involved from adjacent cheek and brow tumors. Only four previous cases have reported primary eyelid tumors. The authors present four cases of primary eyelid margin involvement, which show the variability in clinical presentations. DESIGN: Retrospective case series. The authors present four case studies of lower eyelid margin tumors diagnosed as SSDC. PARTICIPANTS/METHODS: The history of this recently recognized neoplasm is discussed in relation to the cases presented and the role of the ophthalmologist and pathologist in such cases. RESULTS: Primary SSDC of the eyelid margin is a reportedly rare entity. This particular presentation can occur in all age groups; can mimic benign, acanthotic, or basal cell-like tumors; and is usually misdiagnosed initially. This can lead to a delay in definitive treatment for a tumor that classically presents late in its natural history to health professionals. All eight cases of primary eyelid SSDC now reported in the literature have occurred in the lower lid. CONCLUSION: This rare but aggressive tumor is difficult to diagnose from a simple biopsy and may be more common than previously believed. Initial or early diagnosis is important because of unusually invasive characteristics. Recurrence is common and usually leads to extensive tissue loss via direct invasion or subsequent wide resection. Correct histologic diagnosis at the time of initial tumor removal will likely aid in achieving complete excision with fewer recurrences.