Kaposi sarcoma (KS) is an angioproliferative tumor affecting the blood and lymphatic vessels. The human herpesvirus 8 (HHV8) is directly implicated in the development of the disease. Non-AIDS kaposi sarcoma is a rare clinical type of KS and must be distinguished from AIDS-associated KS as it differs in clinical presentation, course, prognosis, and management.

A 73-year old male, Filipino, rice farmer, from Quezon Province, Philippines, presented with a progressive 10-year history of violaceous to hyperpigmented plaques and nodules on all extremities, with associated pruritus and difficulty in performing fine motor tasks. Histopathology showed proliferation of vascular channels and immunohistochemical stains were positive for CD31, CD34, and human herpesvirus 8 (HHV8), consistent with KS. Work-up revealed non-reactive HIV serology with an adequate CD4 count and computed tomography (CT) scan of the chest and abdomen were unremarkable. The case was classified as non-AIDS KS. He was then referred to oncology for chemotherapy with paclitaxel.

This case highlights that KS can occur in non-endemic areas and in immunocompetent individuals. Non-AIDS KS must be differentiated from other types of KS since non-AIDS KS is less aggressive, limited to the skin, and is highly responsive to therapy. It is therefore crucial to correlate clinical and histopathologic findings, utilizing immunohistochemical stains, as histology of KS can mimic benign vascular tumors, and it is crucial to achieve an early and accurate diagnosis. There are currently no treatment guidelines for KS and management aims to decrease morbidity and improve a patient’s quality of life.