Classic Kaposi's sarcoma

ARTICLE

A 23-year-old Albanian HIV-negative male presented with multiple nodular lesions on his left foot. He reported the onset of lesions five months earlier, following a mechanical trauma, as small bluish-red papules that enlarged into nodules. Past medical history revealed a severe burn from an unidentified source on the dorsal surface of his left foot, which occurred in childhood and was treated with a skin graft. The patient was otherwise in good health. There was no history of prior use of cytotoxic drugs or corticosteroids.

At physical examination, several procident reddish-violaceous and rubbery nodules, ranging in size from 0.5 to 1 cm, were present on the dorsal and lateral surfaces of the toes and on the plantar surface of the left foot. On the dorsum, the lesions were adjacent to a large and oval 5 x 7 cm hyperchromic scar resulting from the previous skin graft. Some nodules, which appeared eroded and covered by hemorrhagic crusts or purulent exudate, were tender. The foot appeared considerably edematous (Fig.1).

Laboratory investigations were within normal limits.

Classic Kaposi's sarcoma

Light microscopy

A punch-biopsy from a nodular lesion showed an ill-defined dermal mass, with undifferentiated hyperchromatic spindle cells and, among these, numerous irregular vascular spaces, containing extravasated red blood cells (Fig. 2).

Other investigations

Search for HHV-8 DNA sequences from tissue specimens using a two step polymerase chain reaction was positive.

Histocompatibility antigens typing revealed the following HLA haplotypes: A2, B16, B51(5), DR1, DR2, DR52, DR53, DQ1.

Comment

Kaposi's sarcoma (KS) is a multicentric neoplastic disease characterized by the onset of single or multiple vascular nodules on the skin and mucosae. Visceral involvement may occur in later stages of the disease [1]. The histogenesis of KS is still unclear, and it has been suggested that it may arise from undifferentiated bone-marrow derived cells with features of both endothelial and macrophagic cells [2].

Four clinical subsets of KS are recognized: classic, endemic, iatrogenic-immunosuppressive and AIDS-associated forms. Classic KS predominantly affects the lower extremities of elderly men of Mediterranean, East European or Jewish heritage [1]. Young patients affected by classic KS are rare, but are expected to be observed in areas of high incidence of the disease. In the last five years an increasing number of cases of classic KS in young patients has been reported, with onset ranging from 4 to 29 years [3-5]. All reported patients came from a restricted geographical area, including Italy in 2 cases [3], Turkey in 1 case [4] and Albania in 2 patients of Greek origin [5]. In one of these cases, a 5-year-old child, the 39-year-old mother was also affected [3].

Our patient was positive for HHV-8 DNA sequences and epidemiological studies have indicated that infection with HHV-8 correlates with the risk for KS [6]. Search for HHV-8 DNA has not been performed in most reported cases of early onset KS [3, 5] but in one case [4] it was negative. Also, recent studies have shown positivity for HHV-8 infection in healthy individuals of Mediterranean ancestry [7] suggesting that further investigation is necessary to determine the relationship between KS and HHV-8.

HLA-DR5 haplotype is usually found positive in patients with classic KS, however some patients, including ours, may be negative [8].

The occurrence of KS in areas of trauma, including insect bites, repeated pressure, excoriations, venipuncture, surgical scars and skin grafts, is a known, although uncommon, phenomenon. According to some authors, it may be considered as a Koebner reaction resulting from the release of proinflammatory cytokines, enhancing vascular proliferation [9, 10] and promoting the onset of KS lesions in the traumatized skin site. It is possible that repeated traumas in a localized area may have played an activating role in the onset of the unilateral KS lesions.

References

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2. Masini C, Lesnoni La Parola I, Capuano M, Cattani P, Cerimele F, Fadda G, Cerimele D. Infezione da HHV 8 e sarcoma di Kaposi. G Ital Dermatol Venereol 1999; 134: 315-20.

3. Zurrida S, Agresti R, Cefalo G. Juvenile classic Kaposi's sarcoma: a report of two cases, one with family history. Pediatr Hematol Oncol 1994; 11: 409-16.

4. Eerdem T, Atasoy M, Akdeniz M, Parlak M, Ozdemir S. A juvenile case of classic Kaposi's sarcoma. Acta Derm Venereol 1999; 79: 492-3.

5. Poutoridou I, Katsambas A, Pantazi V, Armenaka M, Stavrianeas N, Stratigos G. Classic Kaposi's sarcoma in two young heterosexual men. J Eur Acad Dermatol Venereol 1998; 10: 48-52.

6. Cattani P, Capuano M, Lesnoni La Parola I, Guido R, Santangelo R, Cerimele F, Masini C, Nanni G, Fadda G, Cerimele D. Human herpesvirus 8 in Italian HIV-seronegative patients with Kaposi sarcoma. Arch Dermatol 1998; 134: 695-9.

7. Viviano E, Vitale F, Ajello F, Perna AM, Villafrate MR, Bonura F, Aricò M, Mazzola G, Romano N. Human herpesvirus type 8 DNA sequences in biological samples of HIV positive and negative individuals in Sicily. AIDS 1997; 11: 607-12.

8. Tzfoni EE, Scherman L, Battat S, Brautbar H. No HLA antigen is significant in classic Kaposi's sarcoma. J Am Acad Dermatol 1993; 28: 118-9.

9. Potouridou I, Katsambas A, Pantazi V, Armenaka M, Vareltzidis A, Stratigos G. Koebner phenomenon in classic Kaposi's sarcoma. Acta Derm Venereol 1997; 77: 481.

10. Micali G, Gasparri O, Nasca MR, Sapuppo A. Kaposi's sarcoma occurring de novo in the surgical scar in a heart transplant recipient. J Am Acad Dermatol 1992; 27: 273-4.