Isolated lichen planus of the conjunctiva

Lichen planus is an autoimmune condition of unknown aetiology affecting the skin and mucous membranes. Classic lesions appear as recurrent, small, discrete, polygonal, flat topped, violaceous, pruritic papules distributed symmetrically on the flexor surfaces of the extremities and trunk. These lesions are often accompanied by lesions of the oral, genital, and rarely, ocular mucosa. Conjunctival lichen planus can lead to irreversible damage to the ocular surface and loss of vision from corneal scarring.¹,² We report two patients with isolated lichen planus of the conjunctiva. The referring ophthalmologists had attributed these ocular findings to a severe dry eye state. The correct diagnosis was made by conjunctival biopsy. Treatment with topical ciclosporin A resulted in suppression of the inflammation and arrest of the progressive cicatrisation.

Case 1

A 57 year old white woman with a diagnosis of secondary Sjögren's syndrome was referred for further management of severe keratoconjunctivitis sicca, which had been refractory to treatment with multiple topical medications, including topical steroids, as well as punctal plugs. The patient had a history of rheumatoid arthritis with crippling deformities in her hands.

On examination, slit lamp biomicroscopy revealed inadequate tear film with debris, severe scar formation of the upper tarsal conjunctiva, moderate bulbar conjunctival hyperaemia, inferior fornix foreshortening, and severe diffuse punctate epithelial keratitis, bilaterally (fig 1). A conjunctival biopsy specimen was harvested from the inferior fornix of the left eye for evaluation. Direct immunofluorescence staining of the specimen revealed a heavy linear fibrinogen deposition along the epithelial basement membrane zone (fig 2). No IgG, IgA, IgM, or C3 deposition was present. A systemic examination of the patient revealed no lesions on the skin, oral, or genital mucosa. A trial of pulsed topical prednisolone acetate 1% was initiated but was quickly tapered and discontinued after a rise in the intraocular pressure. While tapering the topical steroids, the patient was started on ciclosporin A 0.05% eyedrops (Restasis, Allergan Inc) four times daily. The ciclosporin dose was increased to six times daily after the discontinuation of the topical steroids, and within 1 month the inflammation was fully controlled. The ciclosporin was gradually tapered to a maintenance dose of twice daily, on which the patient's cicatrising conjunctivitis has remained quiescent for 20 months, with no evidence of skin or other mucosal involvement.

Figure 1 Patient 1. Significant foreshortening and subepithelial scar formation of the right lower forniceal conjunctiva.

Figure 2 Direct immunofluorescence staining of the lower bulbar conjunctival biopsy specimen from the same patient, demonstrating a heavy, linear deposition of fibrinogen along the basement membrane.

Case 2

An 86 year old white man was referred for further management of keratoconjunctivitis sicca and blepharitis attributed to acne rosacea. The patient's condition had been refractory to treatment with oral tetracyclines combined with topical steroids. His ocular history was remarkable for postoperative endophthalmitis in the left eye following combined cataract extraction and trabeculectomy, necessitating a subsequent penetrating keratoplasty with secondary intraocular lens implantation. The graft failed within a few months of transplantation.

On examination, slit lamp biomicroscopy demonstrated eyelid thickening with irregular margins, marked telangiectasis, and significant meibomian gland dysfunction in both eyes. The bulbar conjunctivae were hyperaemic. Symblepharon formation and foreshortening of lower forniceal conjunctivae were present bilaterally. The corneal epithelium on the right was irregular, with subepithelial and superficial stromal haze. The left eye had a failed corneal graft with 360 degrees of neovascularisation. A conjunctival specimen was harvested from the inferior fornix of the left eye. Direct immunofluorescence staining revealed shaggy linear deposition of fibrin along the epithelial basement membrane zone. No immunoglobulin or complement deposition was present. A detailed medical examination of the patient revealed no lesions on the skin, oral or genital mucosa. A trial of topical ciclosporin A 0.05% drops (Restasis, Allergan Inc) four times daily, combined with prednisolone acetate 1% four times daily, brought the symptoms under control. During the 18 months of follow up, both medications have been successfully tapered, and there has been no progressive cicatrisation.

Comment

Lichen planus is a very common skin disease and the overwhelming majority of the patients have oral lesions as well. However, lichen planus isolated to the conjunctiva is very rare. The cause of lichen planus is unknown. Available evidence points toward a T cell mediated immunological response to an induced antigenic change in the basal membrane zone of mucosa or skin.³ Conjunctival involvement, albeit rare, may be severe, causing cicatrix formation that is clinically indistinguishable from other forms of cicatrising conjunctivitis.¹,² A conjunctival biopsy is the key to the correct diagnosis of the underlying aetiology. An irregular, coarse band of fibrin or fibrinogen deposition in the basement membrane, in the absence of immunoreactants demonstrated with direct immunofluorescein staining, strongly supports a diagnosis of lichen planus.²

Multiple therapeutic options including local and systemic corticosteroids, psoralens with ultraviolet A light, retinoids, ciclosporin, and cyclophosphamide have been recommended.⁴ Ciclosporin A is an immunomodulator that specifically inhibits T lymphocyte proliferation via inhibition of interleukin 2 receptor expression.⁵ Several clinical studies demonstrated favourable results with topical ciclosporin in the treatment of ulcerative or erosive oral lichen planus. Successful treatment of patients with isolated conjunctival lichen planus using topical ciclosporin A 2% in olive oil has also been reported.¹,²

We recommend that lichen planus be routinely included in the differential diagnosis of cicatrising conjunctivitis, as this has important therapeutic and prognostic implications. Topical ciclosporin A may be of benefit in the treatment of selected patients with isolated conjunctival lichen planus.