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Table 1 Subclassification of KS including as described by * Antman and Chang [4] and ā€  Cesarman et al. [5]

From: Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis

Variant

Risk group

Typical presentation

Ā 

Classic*ā€  or sporadic

Middle aged and elderly individuals of Eastern European, Middle Eastern or Mediterranean Origin. Men affected more often than women

Lower limb cutaneous lesions. Mucosal and visceral disease are rare

Not Immunocompromised

Endemic*ā€ 

Individuals (children and adults) from sub-Saharan Africa, who are HIV negative

Adults present with lower limb cutaneous lesions. Children may present with lymph node involvement and lymphedema and have a more aggressive natural history

MSMā€ 

MSM without HIV infection. Often young or middle aged

Usually presents as cutaneous KS (any site). Mucosal and visceral disease are rare

HIV associated KS*ā€ 

HIV infected, especially homosexual or bisexual men. KS risk rises with declining CD4 count

Multiple cutaneous lesions on limb, trunk and face. Mucosal and visceral lesions occur in 15ā€“20% of patients

Immunocompromised

Iatrogenic*ā€ 

Patients receiving immunosuppressive medication. Most common in patients receiving organ transplantation

Usually presents as cutaneous KS (any site). Mucosal and visceral disease can occur

  1. Risk Group; population affected and Typical Presentation; most common presenting clinical features