CONJUNCTIVITIS OF UNKNOWN CAUSE

FOLLICULOSIS

Folliculosis is a widespread benign, bilateral noninflammatory conjunctival condition characterized by follicular hypertrophy. It is more common in children than in adults, and the symptoms are minimal. The follicles are more numerous in the lower than in the upper cul-de-sac and tarsal conjunctiva. There is no associated inflammation or papillary hypertrophy, and complications do not occur.

There is no treatment for folliculosis, which disappears spontaneously after a course of 2-3 years. The cause is unknown, but folliculosis may be only a manifestation of a generalized adenoidal hypertrophy.

CHRONIC FOLLICULAR CONJUNCTIVITIS (Axenfeld's Conjunctivitis)

Chronic follicular conjunctivitis is a bilateral transmissible disease of children characterized by numerous follicles in the upper and lower tarsal conjunctiva. There are minimal conjunctival exudates and minimal inflammation but no complications. Treatment is ineffective, but the disease is self-limited within 2 years.

OCULAR ROSACEA

Ocular rosacea is a common complication of acne rosacea and probably occurs more often in light-skinned people, especially of Irish descent, than in dark-skinned people. It is usually a blepharoconjunctivitis, but the cornea is sometimes also affected. The patient complains of mild injection and irritation. There is frequently an accompanying staphylococcal blepharitis. The blood vessels of the lid margins are dilated and the conjunctiva hyperemic, especially in the exposed interpalpebral region. Less often, there may be a nodular conjunctivitis with small gray nodules on the bulbar conjunctiva, especially near the limbus, which may ulcerate superficially. The lesions can be differentiated from phlyctenules by the fact that even after they subside, the large dilated vessels persist.

Microscopic examination of the nodules shows lymphocytes and epithelial cells. The peripheral cornea may ulcerate and vascularize, and the keratitis may have a narrow base at the limbus and a wider infiltrate centrally. The corneal pannus is often segmented or wedge-shaped inferiorly (Figures 5-16 and 5-17).

Treatment of ocular rosacea consists of the elimination of hot, spicy foods and of alcoholic beverages that cause dilation of the facial vessels. Any secondary staphylococcal infection should be treated (Figure 5-18). A course of oral tetracycline or doxycycline is often helpful, and a smaller maintenance dose may be needed to control the disease.

The disease is chronic, recurrences are common, and the response to treatment is usually poor. If the cornea is not affected, the visual prognosis is good; but corneal lesions tend to recur and progress, and the vision grows steadily worse over a period of years.

PSORIASIS

Psoriasis vulgaris usually affects the areas of the skin not exposed to the sun, but in about 10% of cases lesions appear on the skin of the eyelids, and the plaques may extend to the conjunctiva, where they cause irritation, a foreign body sensation, and tearing. Psoriasis can also cause nonspecific chronic conjunctivitis with considerable mucoid discharge. Rarely, the cornea may show marginal ulceration or a deep, vascularized opacity.

The conjunctival and corneal lesions wax and wane with the skin lesions and are not affected by specific treatment. In rare cases, conjunctival scarring (symblepharon, trichiasis), corneal scarring, and occlusion of the nasolacrimal duct have occurred.

ERYTHEMA MULTIFORME MAJOR (STEVENS-JOHNSON SYNDROME)

Erythema multiforme major is a disease of the mucous membranes and skin. The skin lesion is an erythematous, urticarial bullous eruption that appears suddenly and is often distributed symmetrically. Bilateral conjunctivitis, often membranous, is a common manifestation. The patient complains of pain, irritation, discharge, and photophobia. The cornea is affected secondarily, and vascularization and scarring may seriously reduce vision. Stevens-Johnson syndrome is typically a disease of young people, occurring only rarely after age 35.

Cultures are negative for bacteria; conjunctival scrapings show a preponderance of polymorphonuclear cells. Systemic steroids are thought to shorten the course of the systemic disease but have little or no effect on the eye lesions. Careful cleansing of the conjunctiva to remove the accumulated secretion is helpful, however, and tear replacement may be indicated. If trichiasis and entropion supervene, they should be corrected. Topical steroids probably have no beneficial effect, and their protracted use can cause corneal melting and perforation.

The acute episode of Stevens-Johnson syndrome usually lasts about 6 weeks, but the conjunctival scarring, loss of tears, and complications from entropion and trichiasis may result in prolonged morbidity and progressive corneal cicatrization. Recurrences are rare.

DERMATITIS HERPETIFORMIS

This is a rare skin disorder characterized by symmetrically grouped erythematous papulovesicular, vesicular, or bullous lesions. The disease has a predilection for the posterior axillary fold, the sacral region, the buttocks, and the forearms. Itching is often severe. Rarely, a pseudomembranous conjunctivitis occurs and may result in cicatrization resembling that seen in benign mucous membrane pemphigoid. The skin eruption and conjunctivitis usually respond readily to systemic sulfones or sulfapyridine.

EPIDERMOLYSIS BULLOSA

This is a rare hereditary disease characterized by vesicles, bullae, and epidermal cysts. The lesions occur chiefly on the extensor surfaces of the joints and other areas exposed to trauma. The severe dystrophic type that leads to scarring may also produce conjunctival scars similar to those seen in dermatitis herpetiformis and benign mucous membrane pemphigoid. No known treatment is satisfactory.

SUPERIOR LIMBIC KERATOCONJUNCTIVITIS

Superior limbic keratoconjunctivitis is usually bilateral and limited to the upper tarsus and upper limbus. The principal complaints are irritation and hyperemia. The signs are papillary hypertrophy of the upper tarsus, redness of the superior bulbar conjunctiva, thickening and keratinization of the superior limbus, epithelial keratitis, recurrent superior filaments, and superior micropannus (Figure 5-19). Rose bengal staining is a helpful diagnostic test. The keratinized epithelial cells and mucous debris pick up the stain. Scrapings from the upper limbus show keratinizing epithelial cells.

In about 50% of cases, the condition has been associated with abnormal function of the thyroid gland. Applying 0.5% or 1% silver nitrate to the upper palpebral conjunctiva and allowing the tarsus to drop back onto the upper limbus usually result in shedding of the keratinizing cells and relief of symptoms for 4-6 weeks. This treatment can be repeated. There are no complications, and the disease usually runs a course of 2-4 years.

In severe cases, one may consider 5 mm resection of the perilimbal superior conjunctiva.

LIGNEOUS CONJUNCTIVITIS

This is a rare bilateral, chronic or recurrent, pseudomembranous or membranous conjunctivitis that arises early in life, predominantly in young girls, and often persists for many years. Granulomas are often associated with it, and the lids may feel very hard. Cyclosporine may be effective treatment, as suggested by recent reports.

REITER'S SYNDROME

A triad of disease manifestations-nonspecific urethritis, arthritis, and conjunctivitis or iritis-constitutes Reiter's syndrome. The disease occurs much more often in men than in women. The conjunctivitis is papillary in type and usually bilateral. Conjunctival scrapings contain polymorphonuclear cells. No bacteria grow in cultures. The arthritis usually affects the large weight-bearing joints. There is no satisfactory treatment, though nonsteroidal anti-inflammatory agents may be effective. Corticosteroids will help the iridocyclitis. The disease has been found in association with HLA-B27 antigen.

MUCOCUTANEOUS LYMPH NODE SYNDROME (KAWASAKI DISEASE)

This disease of unknown cause was first described in Japan in 1967. Conjunctivitis is one of its six diagnostic features. The others are (1) fever that fails to respond to antibiotics; (2) changes in the lips and oral cavity; (3) such changes in the extremities as erythema of the palms and soles, indurative edema, and membranous desquamation of the fingertips; (4) polymorphous exanthem of the trunk; and (5) acute nonpurulent swelling of the cervical lymph nodes.

The disease occurs almost exclusively in prepubertal children and carries a 1-2% mortality rate from cardiac failure. The conjunctivitis has not been severe, and no corneal lesions have been reported.

Treatment is supportive only.

10.1036/1535-8860.ch5

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