Classic Kaposi's sarcoma associated with human herpesvirus 8 infection in a 13-year-old male: A case report

Purpose: Classic Kaposi's sarcoma (KS) is rare in children. Although its et iology is not fully understood, human herpesvirus 8 (HHV-8) is present in t he angiogenic lesions. We report an HIV-negative, 13-year-old patient of Si cilian descent with HHV-8-associated classic KS to facilitate the diagnosis and treatment of this entity in children. Experimental Design: DNA was extracted from the skin specimen of the patien t and analyzed via PCR assay and Southern blot hybridization for HHV-8 DNA. The amplified HHV-8 DNA was cloned, sequenced, and compared with the proto type HHV-8-KS330/BAM. Results: The patient presented with purpuric lesions on the distal lower ex tremities and the tip of his nose, associated with thrombocytopenia and leu kopenia, suggesting an immune-mediated cytopenia. While on prednisone, he d eveloped marked vascular proliferation in the groins. Biopsy of the skin le sions showed KS, and HHV-8 was detected in the tissues by PCR. Sequence ana lysis of the amplified DNA was homologous to the prototype HHV-8-KS330/BAM. His HHV-8 strain was the A subgroup, the type associated with Mediterranea n classic KS. Stopping prednisone and treatment with IFN-alpha and IgG resu lted in regression of the groin lesions. Conclusions: This report emphasizes the importance of recognizing classic K S in children and avoiding immunosuppressive therapies in indolent classic KS. The diagnostic and therapeutic strategies were effective and well toler ated.