Case
Advanced · Vascular / Special population
Violaceous skin and oral lesions in newly diagnosed HIV
A 42-year-old man presents with new violaceous lesions on the lower limbs and the palate, having received a positive HIV test the previous week.
Diagnosis
AIDS-related (epidemic) Kaposi sarcoma, ACTG T1 I1 S1 (advanced cutaneous and visceral risk) — treated with antiretroviral therapy plus pegylated liposomal doxorubicin
Learning points
- Human herpesvirus 8 (HHV-8 / KSHV) is the essential driver of Kaposi sarcoma. All four clinical variants (classical Mediterranean, endemic African, iatrogenic / transplant-associated, and AIDS-related / epidemic) share HHV-8 infection of spindle endothelial cells.
- Diagnostic biopsy: dermal proliferation of slit-like vascular channels lined by spindle cells, with extravasated red cells and haemosiderin. Nuclear HHV-8 / LANA (latency-associated nuclear antigen) immunostaining is the confirmatory test.
- ACTG staging (AIDS Clinical Trials Group) is the standard for AIDS-related KS: T (tumour: T0 limited cutaneous / minimal oral; T1 extensive cutaneous, oedema, visceral or extensive oral), I (immune: CD4 ≥/< 200), S (systemic illness).
- Optimisation of antiretroviral therapy is the single most effective intervention — immune reconstitution alone often produces partial or complete regression in mild AIDS-related KS.
- Beware KS-associated immune reconstitution inflammatory syndrome (KS-IRIS) when starting ART in advanced KS — can cause paradoxical worsening, especially in pulmonary disease.
- Systemic therapy for ACTG T1 disease (extensive cutaneous, visceral, lymphoedema or symptomatic): pegylated liposomal doxorubicin is first-line in the UK (paclitaxel is second-line / alternative).
- Iatrogenic / transplant-associated KS — first step is reduction or conversion of immunosuppression (mTOR inhibitor conversion has direct anti-tumour effect on KS).
- Localised cutaneous KS treatment options: intralesional vinblastine, cryotherapy, radiotherapy and electrochemotherapy.