Acquired Elastotic Hemangioma-like Change of the Vulva Associated With Lichen Sclerosus

Abstract

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.