Extraordinary Case ReportCutaneous Angiosarcoma of the Eyelid Mimicking Morbihan DiseaseFerguson, Donna C. MD*; Mawn, Louise A. MD†; Al-Rohil, Rami N. MBBS*Author Information *Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN; and †Department of Ophthalmology and Visual Sciences, Vanderbilt Eye Institute, Vanderbilt University Medical Center, Nashville, TN. Correspondence: Rami N. Al-Rohil, MBBS, 1161 21st Avenue South, C3321B Medical Center North, Nashville, TN 37232 (e-mail: [email protected]). The authors declare no conflicts of interest. The American Journal of Dermatopathology: August 2018 - Volume 40 - Issue 8 - p 617-620 doi: 10.1097/DAD.0000000000001096 Buy Metrics Abstract Background: Cutaneous angiosarcoma presents clinically in numerous ways, and can be mistaken for a different clinical entity, particularly when arising at unusual anatomic locations such as the eyelid. Case Presentation: A 57-year-old woman presented with a 1-year history of eyelid swelling. Concurrent imaging was also suggestive of an edematous process. Multiple superficial biopsies showed nonspecific dermal inflammation and interstitial edema. A diagnosis of Morbihan disease (chronic and idiopathic lymphedema of the eyelid) was rendered, and the patient was treated with compression and local therapy without clinical improvement. Three years after initial presentation, a diagnostic blepharoplasty was performed revealing a deep dermal vascular proliferation composed of anastomosing vascular channels with an atypical endothelial lining. A diagnosis of cutaneous angiosarcoma was ultimately made. Conclusions: This case illustrates a unique presentation of cutaneous angiosarcoma and the implications of different biopsy techniques in acquiring the correct diagnosis. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.