Sir,

Diffuse infiltrative lymphocytosis syndrome (DILS) is a rare manifestation of human immunodeficiency virus (HIV) infection. It is characterised by HIV seropositivity, a CD8+ lymphocytosis, visceral gland infiltration with CD8+ lymphocytes, bilateral parotid gland swelling, xerostomia, and xerophthalmia.1 Peripheral neuropathy, interstitial pneumonitis and a granulomatous panuveitis have also been described in association with this condition.2 We present a case of scleral granuloma occurring in the presence of presumed DILS.

Case report

A 10-year-old girl of Congolese origin, who was HIV positive, gave a 1-month history of a red painful right eye. Examination revealed a 15 mm × 15 mm dark scleral nodule above the cornea which was associated with locally injected scleral feeding vessels, as shown in Figure 1. Anterior segments were otherwise unremarkable but dilated fundoscopy revealed a bilateral periphlebitis, with no haemorrhage or neovascularisation.

Figure 1
figure 1

Scleral nodule with surrounding vascularisation.

Full size image

Systemic examination showed her to be of short stature and to have tinea capitis and tinea unguis, cervical and axillary lymphadenopathy, and bilateral parotid gland enlargement. Serologic analysis revealed absolute CD4+ cell count of 0.38 × 109/l (normal range, 0.30–2.0 × 109/l), absolute CD8+ cell count of 1.04 × 109/l (normal range, 0.30–1.80 × 109/l), and peripheral blood film CD8+ positive percentage raised at 47% (normal range, 19–34%). Full blood count was otherwise normal but C-reactive protein was raised at 26 mg/l. Chest radiography findings were consistent with lymphocytic interstitial pneumonitis. Serology for syphilis, cytomegalovirus, and hepatitis B and C showed neither active disease nor previous infection. Measles serology indicated that she had had previous exposure only. Serum angiotensin-converting enzyme (ACE) level was normal.

She was commenced on a course of topical dexamethasone and highly active antiretroviral therapy (HAART) at the following doses: abacavir 450 mg o.d., lamivudine 200 mg o.d., and efavirenz 300 mg o.d. Over 4 months, the scleral granuloma became smaller, less locally injected and the eye comfortable. Subsequent CD8+ positive percentage improved sequentially to 37%.

Comment

A presumptive diagnosis of DILS is likely given this patient's HIV seropositivity, relative CD8+ lymphocytosis (although this could be due to a relatively lower CD4+), bilateral parotid gland enlargement, and normal ACE. Bilateral panuveitis, retinal periphlebitis, and lacrimal gland involvement are recognised ocular associations in DILS,2, 3, 4 however, a literature search shows no previous report of scleral granuloma occurring in the presence of this syndrome. We appreciate that there was no absolute lymphocytosis, only a reversal of the CD4+ : CD8+ ratio. All cases reviewed in the existing literature have shown absolute lymphocytosis, with numbers much higher than those seen in our patient.

It may be argued that positive tissue diagnosis was required to confirm DILS to show local CD8+ infiltration and to exclude granulomatous pathology, especially given the presence of retinal periphlebitis. However, a negative Mantoux test and a normal chest radiograph and serum ACE excluded both tuberculosis and sarcoidosis. Furthermore, it was deemed inappropriate to subject a 10-year-old girl with resolving parotid swelling to a tissue biopsy. Although typing was not performed in our patient, HLA DR11 has been shown to have a higher association with this condition.5

Although a diagnosis of scleral staphyloma with uveal prolapse was considered, in which case ultrasonic biomicroscopy would have been useful, we thought it was more likely that our patient had developed a scleral granuloma as a result of local CD8+ infiltration and that this regressed with commencement of HAART and topical steroids.

Scleral granulomas have been found in association with different systemic conditions.6, 7, 8, 9, 10 To our knowledge this is the first report of scleral granuloma occurring in the presence of presumed DILS. During HIV infection the immune system becomes dysfunctional because of the coexistence of immunodeficiency and immune hyperactivity, and a disregulated production or activity of cytokines, thereby explaining the development of the DILS. Clinicians need to be aware of this entity, which is gaining in significance.