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Editor,—Kaposi’s sarcoma (KS) is the most common neoplasm in patients with the acquired immunodeficiency syndrome (AIDS). In Europe, approximately 29% of patients with AIDS are affected; the tumour occurs much more frequently among HIV infected homosexual men compared with patients who contracted HIV through different routes of infection.12 Increasing evidence points to the role of the human herpesvirus 8 (HHV8) as a causative infectious agent for KS in immunosuppressed patients. It has not as yet been clarified, however, if HHV8 is required for the development of KS or if the virus preferentially infects and reduplicates within KS.2
Disseminated KS is currently treated with single or multiple agent chemotherapy, immunotherapy, and/or radiation therapy. For limited local disease cryotherapy, surgical excision, or local chemotherapy can be employed.3 We present a case of regression of conjunctival KS under systemic chemotherapy with bleomycin.
A 36-year-old homosexual, clinically asymptomatic male was proved to be HIV positive in June 1994. Within 16 months the patient rapidly developed AIDS (CDC 3) showing severe psoriasis, oral candidiasis, and disseminated mucocutaneous KS. The development of clinical symptoms was paralleled by a drop in CD4+ cell counts from 800 × 106/l to 28 × 106/l during this period of time. Ocular examination showed a 8 × 4 mm, slightly elevated red-coloured lesion of the nasal bulbar conjunctiva of the right eye (see Fig 1), as well as two cutaneous lesions of the upper eyelid of the right eye and the lower eyelid of the left eye, respectively. Further ocular examination was unremarkable. As the patient was disturbed by constant irritation caused by the lesion as well as by its cosmetic appearance, a biopsy was performed which confirmed the diagnosis of conjunctival KS. Antiretroviral treatment was started with zidovudine plus zalcitabine in December 1995. Disseminated KS was treated with 5 mg/day bleomycin injections intramuscularly on 3 consecutive days every 2 weeks. This was initially combined with external beam radiation with ocular shielding for cutaneous lesions of the tip of the nose and the right cheek with a total dose of 12 Gy. No radiation was applied to the right eye. After 12 cycles of this regimen, complete tumour regression of cutaneous and ocular lesions (see Fig 2) occurred. During this period, CD4+ cell counts rose to 110 × 106/l. In December 1996, additional antiretroviral treatment with zidovudine and idinavir was started. No recurrence of KS was observed during the 17 month follow up until May 1997 with current CD4+ cell counts of 168 × 106/l.
With the increasing incidence of HIV infections, patients with ocular manifestations of AIDS are being seen more frequently. In patients with AIDS related KS, ocular involvement of the eyelids or the conjunctiva could be observed in 20% (20/100) of cases.4 Two cases of conjunctival KS as the initial clinical manifestation of AIDS have been reported.56 Conjunctival manifestations of HIV infections are often neglected; they include KS, microvasculopathy, non-specific transient conjunctivitis, dry eye syndrome, and microsporal keratoconjunctivitis.4-6 KS of the conjunctiva appear as flat, reddish lesions, most often located in the lower fornix, which are easily mistaken for chronic subconjunctival haemorrhage, foreign body granuloma, or a cavernous haemangioma. Treatment of conjunctival KS is usually indicated for cosmetic reasons, if the lesion is complicated by haemorrhage or infection, or if it results in visual obstruction.4
Varying forms of treatment have been described; these include local excision with or without adjunct cryotherapy, fluorescein angiography based excision, external beam radiation, local immunotherapy, as well as single or multiple agent chemotherapy.4-8 The major complicating factor in local treatment of conjunctival KS is the relatively high rate of tumour recurrence in 29%–66% of cases.47
Bleomycin as a single agent chemotherapy for mucocutaneous KS has been described by Caumes et al.9 With this therapy, a 74% (52/70) response rate could be achieved. The advantages of bleomycin over other chemotherapeutic agents include its good efficacy and the lack of haematoxicity. The main side effects of bleomycin are adverse skin reactions (for example, flagellated pigmentations and Raynaud’s phenomenon).9 Although two cases of conjunctival KS were included in the series of Caumes et al, no details about their response to therapy are given.9
In this case, bleomycin therapy was accompanied by antiretroviral treatment; further, the regression of KS was paralleled by a rise in the CD4 cell count. A partial response of KS to antiretroviral therapy with zidovudine alone has been reported as well as a complete regression after treatment with indinavir, a new HIV-1 protease inhibitor.10 To what extent antiretroviral therapy might have contributed to the regression of KS in this case cannot be precisely differentiated from the effects of bleomycin above. However, we assume that bleomycin therapy was the major cause of regression of KS since, to our knowledge, complete regression of disseminated mucocutaneous KS with the antiretroviral treatment used in this case alone or in combination has not been reported to date despite the widespread use of antiretroviral agents in patients with AIDS and KS.
In conclusion, a complete and long standing remission of conjunctival KS could be achieved with intramuscular bleomycin therapy. This case is of particular interest in view of the rapid progression of symptoms and drop in CD4 cell count and the unusual involvement of the conjunctiva and both upper eyelids.