The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma.

The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma. Semin Cutan Med Surg. 2016 Mar;35(3):147-52 Authors: Croteau SE, Gupta D Abstract Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy. The classic dermatologic features of these lesions can facilitate diagnosis for the astute provider; however, the absence of or unusual integumentary involvement or presentation in a less common age group (adolescents/adults) poses a diagnostic challenge. Current approaches to the management of KHE/TA are often informed by lesion features such as presence of KMP, extent and location of the tumor, and symptomatology. Evidence-based treatment guidelines are limited. Corticosteroids, vincristine, interferon, multi-agent regimens and newer therapie...
Source: Seminars in Cutaneous Medicine and Surgery - Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research