Case Report of Reactive Angioendotheliomatosis

Abstract

Reactive angioendotheliomatosis (RAE) is a rare, typically self-limiting, angioproliferative disease that is commonly seen in patients with a variety of inflammatory and systemic diseases. RAE can clinically present as ulcerated, single or multifocal, violaceous papules or plaques. We report a case of a 59-year-old male with a complicated cardiac history who presented with biopsy-proven RAE on the thigh. The differential diagnosis of RAE can include Kaposi sarcoma and angiosarcoma, as well as other non-malignant cutaneous disease. These diagnoses must be excluded before correctly diagnosing and treating RAE. We review the common presentation of RAE along with criteria to exclude other possible diagnoses that can resemble RAE.

Hobnail cell change in reactive angioendotheliomatosis, a potential diagnostic pitfall

“…Diseases that can co-exist include vascular abnormalities, hematologic disorders, infectious diseases, monoclonal gammapathies and other systemic diseases (Gleason & Hornick, 2013). In spite of pathogenesis not being fully known, alterations in portal and systemic hemodynamics and response to local hypoxia can be the probable factor behind the disease (Gupta et al, 2020). RAE is a benign proliferation of endothelial cells intraluminal in location on histopathology, which could obstruct the vascular lumen which is also observed in our case (Kim et al, 2002;Mazloom et al, 2017).…”

“…2003). Many patients present with symptoms like fever and weight loss for which systemic examination is done to rule out the underlying systemic disease (Aung et al, 2015;Gupta et al, 2020). Laboratory findings show raised erythrocyte sedimentation rate (Lazova et al, 1996).…”

“…However it is known to occur in relation to the underlying systemic disease (Gupta et al, 2020). It is believed to occur as a result of hypoxia of the tissue which triggers the release of circulating growth factors (Gleason & Hornick, 2013).…”