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Snapshot
  • A 45-year-old man with a history of HIV infection presents to his primary care doctor for a rash. He had been on antiretroviral medications for his HIV infection for years. Six months ago, he lost his job and his insurance and ran out of medication 5 months ago. He had been feeling fine until a week ago when he developed a painless rash on his face, trunk, and legs. He has also had some night sweats and low-grade fevers. On physical exam, he has multiple scattered reddish purple macules and papules. There are also purple nodules on his oral mucosa. A skin biopsy reveals neoplastic spindle-shaped cells and a lymphocytic infiltrate.
Introduction
  • Classification
    • human herpesvirus-8 (HHV-8)
      • an enveloped, linear double-stranded DNA virus
      • causes Kaposi sarcoma, a neoplasm of endothelial cells
  • Epidemiology
    • demographics
      • endemic in Africa
        • causes soft tissue tumors
      • adults > children
    • location
      • skin, lungs, lymph nodes, and gastrointestinal tract
    • risk factors
      • HIV/AIDS 
        • acquired through sexual contact
        • rarely seen in AIDS acquired through injection drug use
      • post-transplant
  • Pathogenesis
    • HHV-8 inhibits tumor suppression pathways
  • Associated conditions
    • diabetes mellitus
    • autoimmune hemolytic anemia
  • Prevention
    • highly active antiretroviral therapy (HAART)
    • annual skin exam in those at risk
  • Prognosis
    • progression is slow
    • recurrence is likely
Presentation
  • Symptoms
    • B symptoms (fevers, night sweats, and weight loss)
    • painless skin lesions
    • may have pain associated with internal lesions
  • Physical exam
    • red or purple lesions of varying morphologies
      • macules and patches
      • papules and plaques
      • nodules
    • common on the face, oral mucosa, legs, and torso
    • lymph nodes may be enlarged
Studies
  • Labs
    • evaluate CD4+ count if AIDS is suspected
  • Skin biopsy
    • neoplastic spindle-shaped cells that form clefts and vascular channels
    • lymphocytic infiltrate
  • Making the diagnosis
    • based on clinical presentation and confirmed with skin biopsy
Differential
  • Bacillary angiomatosis
    • distinguishing factors
      • rash manifests as dark red and purple papules, nodules, and plaques
      • skin biopsy reveals neutrophilic infiltrate
Treatment
  • Management approach
    • HAART in those with AIDS-related disease is mainstay of treatment
    • patients should discontinue any immunosuppressants
  • Medical
    • vincristine
      • indication
        • AIDS-related disease
    • interferon
      • indication
        • AIDS-related disease
    • chemotherapy and radiation therapy
      • indication
        • widespread or rapidly progressing disease
Complications
  • Metastatic progression of disease
 

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