Type | Predominant risk groups | Cutaneous presentation | Visceral involvement | Clinical course |
Classic (sporadic) | Approximately 3:1 male:female ratio Age >60 Mediterranean or Central/Eastern European origin; Middle East | Distal lower extremities | Uncommon | Usually indolent; rarely aggressive and disseminated |
Endemic (African) | Male adults Children of both sexes Equatorial Africa | Various (may be similar to classic or more locally aggressive); lower extremity lymphedema in adults; cutaneous disease often absent in children | Internal organs involved in a subset of adult patients Common (lymph nodes and viscera) in children | Indolent to locally invasive in adults Occasional rapid progression with visceral disease in adults Aggressive in children |
Iatrogenic (immunosuppression related) | Exogenous immunosuppression, especially solid organ transplant Older patients (>50) Use of cyclosporin A | Distal lower extremities; may be disseminated | Relatively common | May regress with modification of immunosuppression May be aggressive |
AIDS associated | Men who have sex with men (resource-abundant countries) Heterosexual males and females (Africa) | Localized or disseminated | Common with poor HIV control | Aggressive or indolent May regress with effective HIV treatment |
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