25671 Nonepidemic variant of Kaposi sarcoma in an immunocompetent HIV-negative male

Abstract

A 30-year-old man with no significant past medical history presented for evaluation of a growth on his nose that appeared 3 months prior. He reported having several female sexual partners in the past with whom he did not consistently use condoms. HIV testing 6 months prior was negative. Physical examination revealed a solitary 7-mm pink to red papule on the nasal dorsum. No other skin or mucosal lesions were identified. There was no cervical, axillary, or inguinal lymphadenopathy. Laboratory workup including CBC, CMP, serum immunoglobulins, and SPEP were unremarkable. HIV 1 and 2, and HTLV-1 and 2 were negative. Shave biopsy revealed a dermal spindle cell proliferation arranged in short intersecting fascicles admixed with plasma cells and occasional mitotic figures. Immunohistochemical studies showed spindled cells stained with CD34. CD31 and HHV8 staining were also positive. Kaposi sarcoma (KS) is a rare angioproliferative disorder associated with infection with human herpes virus 8 (HHV-8). There are 4 recognized epidemiologic forms: classic, endemic, epidemic, and iatrogenic. A recently described fifth type, nonepidemic KS, occurs in a rare subset of individuals who do not fit other classifications. This includes HIV-negative individuals without detectable cellular or humoral immunodeficiency. This subset has been described as clinically similar to classic KS with limited disease, but occurring in younger men. Although nonepidemic KS is generally indolent with a good prognosis, secondary lymphoproliferative disorders, including Castleman disease and lymphoma, have been reported. This report highlights a case of nonepidemic KS and discusses important relevant diagnostic and management considerations.