Iatrogenic Kaposi's sarcoma following steroid therapy for nonspecific interstitial pneumonia with HHV-8 genotyping

Abstract

We present a case of iatrogenic Kaposi's sarcoma (KS) in a 64-year-old, human immunodeficiency virus (HIV)-negative woman with nonspecific interstitial pneumonia (NSIP) following steroid therapy. She suffered from longstanding exertional dyspnea, and was diagnosed to have NSIP by a thoracoscopic lung biopsy. After 4 months of steroid therapy, multiple purplish nodular or patchy skin lesions developed throughout the entire body. Microscopically, the skin lesions, consisting of closely packed spindle cells forming slit-like vascular structures, were diagnosed as typical KSs. The tumor cells were positive for CD34, factor VIII-related antigen, and human herpesvirus 8 (HHV-8) immunostainings. Using fresh tumor tissue and peripheral blood, polymerase chain reactions revealed the presence of HHV-8. Phylogenetic analysis of HHV-8 ORF K1 genes disclosed that the strain belonged to the subtype II/C and had the M allele at the right-hand side of the genome. To the best of our knowledge, this case report is the first documentation of iatrogenic KS associated with NSIP. A brief review focusing on the HHV-8 genotypes of iatrogenic KS is also presented.