On this page
Referral and warning signs
When to refer
Refer ALL suspected or confirmed KS cases to specialist care for diagnosis confirmation, staging, ART optimization, and consideration for local or systemic therapy.
Red flags
- Rapidly proliferating or ulcerating skin lesions
- Suspected pulmonary involvement (cough, dyspnoea, haemoptysis)
- Suspected significant GI involvement (bleeding, pain)
- Severe lymphoedema
- Signs of KS-IRIS after starting ART
Signs and symptoms
- Skin Lesions: Most common. Red, purple, brown, or black macules, papules, plaques, or nodules. Often multifocal. May have oedema.
- Oral Lesions: Palate (esp. hard palate), gums, tongue.
- Lymphadenopathy.
- Visceral Involvement: Gastrointestinal (often asymptomatic, can bleed), Pulmonary (cough, dyspnoea, haemoptysis, effusions - poor prognosis).
- Lymphoedema: Significant swelling (lower limbs, face, genitalia).
Risk factors
- Human Herpesvirus-8 (HHV-8 / KSHV) infection (necessary cause).
- Immunosuppression: HIV/AIDS (Epidemic KS), Post-transplant (Iatrogenic KS).
- Older men of Mediterranean/Eastern European descent (Classic KS).
- Endemic African KS (HIV-negative, can be aggressive/lymphadenopathic).
Diagnosis
Clinical assessment
Suspect based on characteristic violaceous skin/oral lesions, especially in HIV-positive individual or risk group. Assess extent of disease.
Investigations
- Skin Biopsy: Essential for diagnosis. Punch or excisional. Histopathology (spindle cells, vascular slits, RBCs). Immunohistochemistry for HHV-8 (LANA-1 stain).
- HIV Testing: Mandatory.
- CD4 Count and HIV Viral Load (if HIV positive).
- Staging Investigations: Thorough skin/oral exam. Chest X-ray. Consider CT Chest/Abdomen/Pelvis or endoscopy if visceral involvement suspected.
Diagnostic criteria
Histopathological confirmation from biopsy.
Management
Non-pharmacological management
- Referral to specialist care (Oncology/Dermatology/HIV clinic).
- Optimize HIV Management (for AIDS-related KS): ART is cornerstone.
- Local Therapies (limited disease): Cryotherapy, Intralesional chemo, Topical therapy, Radiotherapy.
- Management of Lymphoedema: Compression, skin care.
Pharmacological treatment
- Antiretroviral Therapy (ART): Essential first-line for HIV+ KS.
- Systemic Chemotherapy: Indicated for widespread/progressive cutaneous disease, symptomatic visceral disease, KS-IRIS, failure of ART alone. Preferred: Liposomal anthracyclines or Paclitaxel. Older: ABV.
Monitoring
Monitor KS lesions. Monitor immune status (CD4/VL). Monitor for side effects. Screen for visceral disease if indicated.
Complications
- Cosmetic disfigurement
- Pain
- Lymphoedema
- Ulceration, secondary infection
- Bleeding (GI or pulmonary)
- Organ dysfunction (e.g., respiratory failure)
- Complications of chemotherapy/ART
Prevention
- Prevent HIV infection.
- Early initiation of ART in HIV+ individuals.
- Prevent HHV-8 transmission (hygiene).
Patient education
- Explain KS diagnosis and link to HHV-8 and immunosuppression (esp. HIV).
- Crucial role of ART adherence for HIV+ patients.
- Treatment options and goals.
- Potential side effects.
- Importance of regular follow-up.
- Address cosmetic concerns and psychosocial impact.
Uganda-specific notes
Very common AIDS-defining illness before widespread ART; still prevalent. Endemic form also occurs. ART is primary treatment for HIV-associated KS. Access to systemic chemotherapy (liposomal agents) and radiotherapy limited.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 12, Section 12.3.2
- Uganda Clinical Guidelines (UCG) 2023
Chapter 3 (HIV Management relevant)