Rosai-Dorfman Disease Presenting as Peripheral Vascular Insufficiency

Rosai-Dorfman (-Destombes) disease (RDDD) is a rare idiopathic disorder of histiocyte proliferation, usually involving lymph node stations. The most common clinical finding is a bilateral cervical lymphadenopathy, fever, and weight loss. Arterial or venous structures are notably not involved. We hereby present a case of a 78-year-old Caucasian man, presenting with symptoms of progressive arterial insufficiency and right lower-limb edema, along with a nonpulsatile mass at the middle third of the thigh. Initial diagnostic hypothesis was a superficial femoral artery aneurysm thrombosis with a secondary postcompressive superficial femoral vein thrombosis. Duplex examination showed right superficial femoral arterial and venous thrombosis, along with a hypoechogenic mass causing compression of the neurovascular bundle. Suspecting a connective tissue sarcoma, computed tomography scan was performed after combined en bloc removal of the mass along with femoral artery and vein and prosthetic reconstruction of vascular continuity. Histopathology diagnosis was connective tissue RDDD. The atypical presentation of this rare syndrome induces us to include in differential diagnosis, among other more common forms of external compression of the neurovascular bundles, even rare conditions such as these, which generally only involve lymphatic stations.