Kaposi's sarcoma classic C46.-

Rare multifocal angioproliferative neoplasia occurring primarily in the skin and secondarily also in other organs. Classical Kaposi's sarcoma occurs spontaneously in HIV-negative men of Mediterranean origin of older age (>75 years).

Endemic in Mediterranean peoples (so-called Mediterranean variant of Kaposi's sarcoma) as well as in Jewish enclaves.

Infectious disease caused by HHV-8; in a Finnish (!) population with familial classic KS a mutation in the STAT4 gene (see STAT below) was detected.

m:f = 18:1; first manifestation > 75 years (in contrast to the epidemic KS; here the age of onset of the disease is 30 years) In larger collectives, an average age of 75.8 years was given for the first manifestation of the disease.

>90% of lesions are found on the lower extremity, especially on the lower legs. Occasionally also on the upper extremity and on the face

Multiple, uni- or bilaterally occurring, initially mostly painless red or red-blue spots, which develop over the course of months/years into red flat plaques and later into red or blue-red nodules. Mostly occurring in combination with lymphedema. Confluence to larger (>10.0cm) plaques. Erosions, excoriations and ulcers possible. Infestation of internal organs often goes unnoticed.

S.u. Kaposi sarcoma (overview)

Several therapy modalities are described as successful.

In the case of localized infestation, surgery can be performed (excision, ablative laser, curettage). At the same time a compression therapy of the US is recommended.

Chemotherapy is recommended for disseminated infestation.

Moderately good forecast with decades of progress. Metastasis in lung and lymph nodes possible.

1. Aavikko M et al (2014) Whole-Genome Sequencing Identifies STAT4 as a Putative Susceptibility Gene in Classic Kaposi Sarcoma. J Infect Dis 211:1842-1851
2. Almohideb M et al ()2013) Familial classic Kaposi sarcoma in two siblings: case report and literature review. J Cutan Med Surg 17:356-361
3. Díaz-Ley B et al (2015) Classic Kaposi's sarcoma treated with topical rapamycin. Dermatol Ther 28:40-43
4. Laresche C et al (2014) Kaposi's sarcoma: a population-based cancer registry descriptive study of 57 consecutive cases diagnosed between 1977 and 2009 Int J Dermatol 53:e549-554
5. Renteria AS et al (2013) A Unique Case of Classic Kaposi's sarcoma restricted to the toes. J Dermatol Case Rep 30: 97-100
6. Tourlaki A et al (2015) Recommended surgery of Kaposi's sarcoma nodules. J Dermatologist Treat 26:354-356
7. Trevisan Fet al. (2013) Classic Kaposi's sarcoma treated with elastic stockings and outpatient follow-up of a 90-year-old patient An Bras Dermatol 88 (6 Suppl 1): 200-2002