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Superior Limbic Keratoconjunctivitis (SLK)

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Superior Limbic Keratoconjunctivitis (SLK) Eye (1989) 3,180-189 Superior Limbic Keratoconjunctivitis (SLK) J. D. NELSON Minnesota Summary Superior limbic keratoconjunctivitis (SLK) is a disease characterised by inflam­ mation of the upper palpebral and superior bulbar conjunctiva, keratinisation of the superior limbus and corneal and conjunctival filaments. It may be associated with other diseases such as keratoconjunctivitis sicca, hyperthyroidism and hyper­ parathyroidism. Although its exact aetiology is unknown, a mechanical hypothesis seems most attractive. Treatment is aimed at altering the abnormal mechanical interaction of the upper lid and superior corneal limbus. Superior Limbic Keratoconjunctivitis is a gested its present name, superior limbic ker­ disease of unknown aetiology which is charac­ atoconjunctivitis (SLK).I terised by (1) inflammation of the superior tarsal Aetiology conjunctiva; The exact cause of SLK is unknown, although (2) inflammation of the superior bulbar infectious,· immunologic,S and mechanical conjunctiva; aetiologies have been postulated.' Ker­ (3) fine punctate staining of the cornea adja­ atoconjunctivits sicca has also been suggested cent to the superior corneal limbus; as a cause. A viral aetiology is unlikely as viral (4) proliferation of superior limbic epithelial cultures have been universally negative and cells; no viral particles have been seen by transmis­ (5) filaments of the upper cornea and sion electron microscopy.7 Although an auto­ limbus. I immune aetiology has been proposed due to The disease was probably firstnoted in 1953 the association of thyroid abnormalities with by Braley and Alexander who described SLK,s.B this is unlikely as there are minimal patients with superficialpunctate keratitis and inflammatory changes seen by light micro­ filaments.2 The disease was further described scopy, no immunoglobulin deposition by by Thygeson in 1961' in 12 patients with a direct immunofluorescence, no circulating filamentary keratitis limited to the superior antibodies by indirect immunofluorescence, cornea associated with inflammation of the no eosinophils and the poor response to superior bulbar conjunctiva. Thygeson and topical cortical steroids. 9 Keratoconjunctivitis Kimura further elaborated on the disease in sicca (as defined by an abnormal Schirmer 1963.4 However, it was not until later in 1963 test) has been noted in approximately one­ that Frederick H. Theodore gave the first quarter of patients with SLKlO-13 (Table I). complete description of the disease and sug- Wright has proposed that the superior pal- From Departments of Ophthalmology, St Paul-Ramsey Medical Center and Ramsey Clinic, St Paul, Minnesota and the University of Minnesota, Minneapolis, Minnesota. Correspondence to: J. Daniel Nelson, MD, Department of Ophthalmology, St Paul-Ramsey Medical Center, St Paul, MN, 55101, USA. SUPERIOR LIMBIC KERATOCONJUNCTIVITIS 18 1 Table 1 Summary of clinical findingsin patients with SLK Author CorwinlV Cher5 Wright" Passonsll Ude{fl2 Ohashi/! Nelson Mean % No. of patients 22 10 23 10 11 12 5 * Age 30--55 years 19 8 8 - 12 5 88.1 Female 18 8 8 8 7 II 5 69.9 Bilateral 5 19 9 3 5 69.5 * Filaments 15 2 12 2 - 4 50.0 Thyroid Disease 5 6 5 3 It 33.3 Decreased Schirmer test 0 4 6 5 51 24.2 * Specific information as to these findings were not reported. t One other patient had hyperparathyroidism due to a parathyroid adenoma. pebral conjunctiva is responsible for initiating SLK. At the present time, a mechanical the pathological process which results in aetiology seems most attractive. SLK.6 He suggested that a chronically inflamed upper lid might cause a disturbance Clinical Features of the normal maturation of cells of the bulbar Patients with SLK usually complain of irrita­ conjunctiva giving rise to the symptoms and tion, or a gritty sensation, redness and a signs of SLK. The difficulty with this hypoth­ mucoid discharge from the eye. Other symp­ esis is that the amount of superior tarsal toms include foreign body sensation, burning inflammation is relatively mild and it is diffi­ and photophobia. These symptoms tend to vary in severity, with time and activity, and, cult to identify whether the superior bulbar or generally coincide with clinical signs. Discom­ palpebral conjunctiva is the initial site of fort is greatly increased by the presence of inflammation.7 Donshik, etal.,7 have sug­ limbal or corneal filaments. gested that the abnormality resides in the SLK has been reported to occur in all age superior bulbar conjunctiva. groups but is more common in patients Ostler has proposed a mechanical hypoth­ between the ages of 30-55 years and women esis.14 He suggests that the various findings of are more frequently affected than men SLK can be explained by the movement of the (Table I). This disease is usually bilateral but upper eye lid which, due to a thyroid abnor­ may occur unilaterally and asymmetrical mality, exophthalmos, or specific or non­ involvement is not unusual. TenzelH first sug­ specific chronic inflammation, is in tight gested an association of SLK and thyroid apposition to the superior bulbar conjunctiva. disease which was later confirmed by Cher. 5 The superior bulbar conjunctiva, which may Since then others have found thyroid disease be lax due to congenital or ageing processes, is in 20-50 per cent of patients with SLK subjected to constant abnormal movement as (Table I). the eyelid opens and closes. This abnormal The classical findings of SLK, as initially movement results in a chronic irritation of the reported by Theodore, are listed in Table II. bulbar conjunctiva due to mechanical factors. These findingscan be divided into those of the He also suggests that the papillary hyper­ superior palpebral conjunctiva, the superior trophy and cellular infiltration of the superior bulbar and limbal conjunctiva and the cornea. palpebral conjunctiva arise due to these There is a papillary reaction of the superior mechanical factors. Supporting this hypo­ palpebral conjunctiva which consists of many thesis are the findings that removal, or ther­ small papillae (Fig. 1). The inferior palpebral mal cauterisation, of the abnormal bulbar conjunctiva is strikingly normal. The superior conjunctiva or treatment of the upper pal­ bulbar conjunctiva is hyperemic and thick­ pebral conjunctiva with silver nitrate result in ened and may appear lusterous. The hyper­ improvement of the signs and symptoms of aemia and thickening extend in a 10 mm arc 182 J. D. NELSON Table II Classicalfindings in patients with SLKJ·3 seen in the Department of Ophthalmology at the University of Minnesota. Table III sum­ Age: 20--67 years Sex incidence: Women> men marises the findings in these patients. Inter­ Duration: 1-10 years estingly, one patient, who presented with Prognosis: Eventually clears classical SLK, was found to have a very ele­ Usually bilateral vated serum calcium and parathyroid hor­ Papillary reaction of superior palpebral conjunctiva mone. Subsequently she was found to have a Superior bulbar conjunctival injection Fine punctate staining of superior cornea and very large parathyroid adenoma. Surgical conjunctiva removal of the adenoma resulted in total Rose Bengal staining of superior cornea and resolution of her symptoms and clinical conjunctiva findings. Proliferation of superior limbus to varying degrees Superior corneal and conjunctival filaments Decreased Schirmer test in some patients Histology Occasional corneal hypesthesia By light microscopy, biopsy specimens of Favourable response to 0.5 per cent silver nitrate in superior bulbar conjunctival specimens show some cases marked keratinisation of the epithelial cells and acanthosis7.l5 (Fig. 4). Some epithelial cells are swollen and in various stages of from the superior limbus centred at 12:00 degeneration. Few goblet cells are present. 6 o'clock toward the superior rectus muscle, and may stain with rose bengal, and less so with fluoresceinor Alcian Blue (Fig. 2). Occa­ sionally, a pseudomembrane! or a subcon­ junctival haemorrhage may be seen.6 The superior one-third of the c9rnea is involved with a finepunctate keratopathy which stains with rose bengal and fluorescein. In about one-half of patients (Table I), multiple fila­ ments may be found on the cornea, superior bulbar and palpebral conjunctiva (Fig. 3). Decreased corneal sensation has been observed in one patient with many filaments, which returned to normal when the filaments disappeared.! Acquired 'with-the-rule' astig­ Fig. 1. The superior palpebral conjunctiva of a patient with SLK. Note the increased vascularity and matism which disappeared with resolution of the finepapillary reaction of the palpebral conjunctiva. SLK has been reported.5 Decreased tear secretion (as evidenced by an abnormal Schirmer test) may be seen in approximately one-quarter of SLK patients (Table I). Viral cultures are universally negative, although staphylococci have been isolated on bacterial cultures.l The natural history of the disease involves a chronic, prolonged course with gradual, com­ plete clearing. Short remissions are seen during the early course of the disease, which gradually lengthen until the disease disap­ pears. Occasionally treatment of underlying thyroid disease, if present, will result in Fig. 2. Fluorescein staining of the superior limbus improvement of the signs and symptoms of and bulbar conjunctiva of a patient with SLK. Note the SLK. fine punctate staining involving the superior cornea, During 1984, five patients with SLK were limbus and conjunctiva. SUPERIOR LIMBIC KERATOCONJUNCTIVITIS 183 Fig. 3.
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