Non–AIDS-Related Kaposi Sarcoma Involving the Tarsal Conjunctiva and Eyelid Margin FREE

Kaposi sarcoma (KS) is a low-grade, multicentric vascular neoplasm that has been described in 4 clinical forms: classic, African, immunocompromised, and epidemic (AIDS-related) KS. Though ocular involvement by KS is rare, it is typically observed in the epidemic form and is most commonly seen as conjunctival or eyelid lesions in the setting of multicentric disease. We describe a patient with ocular KS without any of the risk factors previously described for the 4 clinical subtypes of KS.

A 62-year-old Hispanic man was seen at the Long Beach Veterans Affairs Medical Center in California with a 12 × 10-mm hemorrhagic, telangiectatic, papillomatous lesion on his upper eyelid margin and tarsal conjunctiva, as well as an adjacent 3 × 4-mm broad-based lesion on the upper eyelid margin (Figure 1). The patient reported that the larger mass grew from a pinpoint, red lesion on his upper eyelid to its current size over the course of 1 month. He stated that the lesion occasionally bled and was irritating to the eye. His medical history was significant for hypertension and his ocular history included chronic open-angle glaucoma. Social history revealed occasional alcohol use and a remote history of smoking in his teenaged years. Based on the clinical appearance, a presumptive diagnosis of pyogenic granuloma was made, and the lesion was injected with intralesional steroids (0.1 mL of triamcinolone acetonide [Kenalog] 40 mg/mL). On follow-up examination 3 weeks later, the pedunculated mass had grown to 15 × 13 mm, and an excisional biopsy was performed. The satellite lesion was not excised. The pathologic findings from the biopsy revealed KS with areas of inflammatory cellular infiltrate (Figure 2), and immunohistochemical stains revealed the presence of human herpesvirus 8 (HHV-8) (Figure 3).

The patient was subsequently treated with cryotherapy to the base of both lesions at the eyelid margin and tarsal conjunctiva. Following treatment, the satellite lesion regressed and the patient has not demonstrated any recurrence of this lesion or development of other cutaneous lesions during the 8-month follow-up period.

On a second review of his social history, the patient admitted having had 2 episodes of unprotected heterosexual encounters over the past 5 years but denied any intravenous drug abuse. He had never received immunosuppressive therapy. Findings from a complete blood cell count and chest radiograph were normal and results from a human immunodeficiency virus (HIV) (enzyme-linked immunosorbent assay) test were negative. His internist also ordered a colonoscopy, the results of which were normal. The patient did not demonstrate any lesions on his trunk or lower extremities suggestive of multicentric KS.

The classic form of KS typically occurs in elderly men of Jewish, Italian, or Greek descent and is seen on the lower extremities as violaceous macules and plaques. Although visceral involvement is uncommon, lower extremity venous stasis and lymphedema can occur. Risk factors associated with classic KS include topical steroid use, infrequent bathing, asthma, and a history of allergy in men.¹ The endemic form of African KS has both cutaneous and lymphatic forms: the cutaneous form is seen as nodules on the lower legs and feet in middle-aged men who are seronegative for HIV, while the lymphatic form occurs in children younger than 10 years and can be rapidly fatal.^{2 - 5} Kaposi sarcoma may also occur in the setting of exogenous immunosuppression, typically after solid-organ transplantation; KS lesions in these patients resemble those seen in the classic form of the disease, but the lesions may be distributed in other areas, including the visceral cavity.^{6 - 7} The epidemic of HIV-related KS may be the AIDS-defining diagnosis in up to 10% to 15% of patients. In Africa, this form of KS is commonly seen in heterosexual adults, while in developed countries such as the United States, afflicted patients are typically homosexual males.⁸ The epidemic form of KS is the most aggressive, frequently occurring in multicentric fashion with involvement of skin, mucosal membranes, lymph nodes, and the visceral cavity.

We report a rare case of ocular KS in a Hispanic patient without HIV or other forms of immunosuppression. Although it is possible that our patient has an underlying immune deficiency that has not been detected, his normal white blood cell count, negative serologic testing for HIV, lack of opportunistic infections, and negative review of systems suggest a competent immune status at the time we saw him. Ocular involvement by KS in a patient who is serologically negative for HIV is extremely rare. In 1994, Ron et al⁹ reported a case of conjunctival KS in an 83-year-old Jewish male patient of East European origin; this was presumed to be an atypical manifestation of classic KS as the patient later developed multifocal cutaneous lesions in the lower extremities. An association with non-AIDS KS and visceral lesions has been reported in the past as well. Kalinske and Leone¹⁰ described a patient with eyelid and conjunctival KS, who was found to have a gastrointestinal malignant neoplasm. However, the findings from a colonoscopy performed on our patient were normal. Our case is unique as our patient had localized ocular involvement without evidence of immunosuppression or the typical background or risk factors suggesting the classic or endemic forms of KS.

Through DNA analysis, HHV-8 has been identified as the causative, etiological agent for KS.¹¹ Infection by HHV-8, which is also known as KS-associated herpesvirus, is thought to precede the development of the tumor in all clinical variants of KS. This virus has also been associated in 2 AIDS-related lymphoproliferative disorders: primary effusion lymphoma and the plasma-cell variant of multicentric Castleman disease¹² as well as the vascular tumor recurrent angiolymphoid hyperplasia with eosinophilia.¹³

In summary, ocular KS should be suspected in patients demonstrating the clinical features of this vascular neoplasm without the previously described risk factors. Even in atypical cases, the diagnosis of KS can be confirmed by its distinctive histopathologic features and the demonstration of HHV-8 in the tissue specimen.

Correspondence: Dr Kim, Department of Ophthalmology, University of California, Irvine, 118 Med Surge I, Irvine, CA 92697-4375 (jwkim3@uci.edu).

Financial Disclosure: None reported.