Classic Kaposi sarcoma presenting as elephantiasis nostras verrucosa

Kaposi sarcoma is a malignant disease that originates from the lymphatic system. Different epidemiological, clinical and histopathological variants of this neoplasm have been identified. Classic Kaposi sarcoma is one of the four main clinico-epidemiologic variants. Cutaneous lesions vary from pink patches to dark violet plaques, nodules or polyps, depending on clinical variant and stage. Kaposi sarcoma with elephantiasis is reported in the context of AIDS. An 82-year-old male presented with a 2-year history of progressive verrucous skin changes and non-pitting edema consistent with elephantiasis nostras verrucosa (ENV), secondary to Kaposi’s sarcoma. Past medical history, physical examination, lab tests and imaging ruled out common causes of ENV and anti-HIV antibody test was negative. Classic Kaposi sarcoma was confirmed on biopsy. To the best of our knowledge, this study reports the first case of elephantiasis nostras verrucosa in an HIV-negative patient with classic Kaposi sarcoma.