BACKGROUND: Epitheloid hemangioendothelioma is a rare malignant tumor which can involve bones, liver, lungs, kidneys, deep soft tissue, muscles, dermis, and central nervous system. Multifocal disease occurs in 10% of the cases. The clinical presentation results from occlusion of small blood vessels due to the disease itself or as a paraneoplastic syndrome. CASE REPORT: We present a patient with symptoms suggesting systemic vasculitis (ESR and CRP elevated, weight loss, arthralgia, livedoid rash, and skin ulcerations) who finally was diagnosed having a disseminated epitheloid hemangioendothelioma when PET scan revealed hypermetabolic multifocal skeletal and soft tissue lesions. DISCUSSION: Diseases mimicking systemic vasculitis and the value of PET in this setting are discussed.
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Haap et al. (2005) studied this question.