Lichen planus is a relatively common mucocutaneous disorder of unknown etiology that is thought to be immunologically mediated. It is a chronic disorder characterized by periods of exacerbation and remission. Lichen planus has variable clinical manifestations, including the skin, oral mucosa, genital mucosa, nails, and scalp. The characteristic lesions have a well-established clinical appearance and histological features that help in establishing the diagnosis.
1
Ocular involvement may occur in patients with lichen planus. Although involvement of the eyelids, lacrimal ducts, conjunctiva, and cornea has been described in patients with lichen planus,2
scleritis has not been reported in such patients. We report a case of scleritis diagnosed in a patient with lichen planus.Case Report
A 46-year-old female presented with redness, discomfort, and pain on ocular movement in the right eye. Review of systems revealed history of pruritic skin lesions of the elbows and knees. Ocular history revealed epiphora, for which she had irrigation of the nasolacrimal ducts, which revealed patent passages. She had been recently diagnosed with lichen planus by her dermatologist, for which she was given local steroid skin creams.
On eye examination, her uncorrected visual acuity was 20/25 OU. The Schirmer test without anaesthesia revealed 15 mm of wetting in the right eye and 13 mm of wetting in the left eye after 2 minutes. Slit-lamp biomicroscopy of the right eye revealed normal lids and conjunctiva. The sclera was injected temporally and inferiorly, with marked tenderness; the cornea was clear; the anterior chamber was quiet; the pupil was round, regular, and reactive; and the lens was clear. Slit-lamp biomicroscopy of the left eye revealed normal findings.
The diagnosis of scleritis was confirmed by anterior segment optical coherence tomography, which was done on the sclera of the right eye. It revealed intrascleral edema and increased sclera thickness consistent with the diagnosis of scleritis. Other causes of scleritis were excluded by extensive questionnaire and negative laboratory work-up. Complete blood count revealed slightly increased neutrophils; erythrocyte sedimentation rate was normal; antinuclear antibodies (ANA) were elevated; anti–cyclic citrullinated peptide (anti-CCP) antibody testing revealed negative results; antineutrophil cytoplasmic antibodies (c,p ANCA) were negative; tuberculin skin testing was negative; and uric acid levels were normal.
She was treated with topical prednisolone acetate eye drops and oral piroxicam 20 mg tab. After 9 days, she came for follow-up and was found to have markedly improved symptoms and signs of scleritis. The patient was followed up for 3 weeks with complete resolution of scleritis. Oral piroxicam was discontinued, and topical prednisolone acetate eye drops were tapered.
Discussion
Ocular involvement may occur in patients with lichen planus. It may affect the eyelids in the form of pruritic violaceous papules.
2
Ocular inflammatory changes with cellular infiltration, fibrosis, and basement membrane thickening in patients with lichen planus may lead to lacrimal duct stenosis and lacrimal canalicular obstruction.3
Conjunctival involvement has been described in lichen planus in the form of conjunctival inflammation, cicatrization, and subepithelial scarring.2
Lichen planus may be also associated with keratitis, keratoconjunctivitis, and persistent epithelial defects, leading to noninfectious or infectious corneal ulceration.
2
Our patient developed severe scleritis in the right eye shortly after a diagnosis of lichen planus was made by her dermatologist. She gave a history of pruritic skin lesions (initially diagnosed as psoriasis and eczema and later as lichen planus) and epiphora. She reported irrigation of both nasolacrimal ducts, which revealed patent passages. She might have had canalicular or ductal stenosis, which has been previously reported in association with lichen planus. Other serious and more common underlying pathologies were ruled out, particularly rheumatoid arthritis (negative anti-CCP) and Wegener’s granulomatosis and polyarteritis nodosa (negative c,p ANCA). In addition, serum uric acid levels were normal, and there were no clinical features of systemic lupus erythematosus.The long-term prognosis in patients with lichen planus is variable. Generalized lichen planus tends to heal faster than other variants but has a greater likelihood of relapse. In patients with cutaneous lichen planus, the lesions resolve within 6 months to a year; however, the hypertrophic variant of the disease tends to persist for years if not properly treated. Improperly treated reticular oral lichen planus has a chronic course and may be progressive in nature without complete resolution. Lichen planus may have a recurrent pattern.
2
Lichen planus is a T-cell-mediated autoimmune disease. Inflammatory cells involved in this process consist of T-helper and T-cytotoxic lymphocytes and other inflammatory cells. T-cell activation plays a central role in the pathogenesis of lichen planus. Normally, the human sclera contains few or no macrophages, Langerhans’ cells, neutrophils, or lymphocytes. After scleral inflammation, there is a marked increase in T-helper lymphocytes with a high T-helper: T-suppressor ratio. These findings suggest that T lymphocytes may play a role in some forms of scleritis.
4
On the other hand, ANA may play a role in the pathogenesis of scleritis in patients with lichen planus. Serum ANA were more frequently and significantly observed in patients with some forms of lichen planus.5
, 6
Zierhut et al. found that 28% of their patients with scleritis had positive ANA.7
These findings may suggest an autoimmune disposition related to collagenosis.7
In our patient, ANA may have contributed to the development of scleritis. The cutaneous lichen planus lesions in our patient responded dramatically to the local steroid creams. Similarly, scleritis responded to anti-inflammatory therapy, suggesting common underlying pathologic mechanisms. However, the exact mechanism of how lichen planus might have affected the sclera rather than the conjunctival or corneal epithelium is yet to be identified.Lichen planus should be included in the differential diagnosis of cases that may underlie scleritis in the absence of other systemic disease associations. This is particularly important in patients presenting with other ocular features and skin lesions suggestive of lichen planus. It is important to diagnose and treat both ocular and systemic disease efficiently to avoid potentially serious complications.
Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
References
- Oral lichen planus: an overview.J Pharm Bioallied Sci. 2015; 7: S158-S161
- Cutaneous and mucosal lichen planus: a comprehensive review of clinical subtypes, risk factors, diagnosis, and prognosis.Sci World J. 2014; 2014: 742826
- Lacrimal canalicular duct scarring in patients with lichen planus.Arch Dermatol. 2012; 148: 224-227
- Immunopathology of scleritis.Ophthalmology. 1991; 98: 472-479
- Modulation of serum antinuclear antibody levels by levamisole treatment in patients with oral lichen planus.J Formos Med Assoc. 2011; 110: 316-321
- Antinuclear antibodies in patients with lichen planus.Exp Dermatol. 1997; 6: 54-56
- [Autoantibody pattern in scleritis and episcleritis].Ophthalmologe. 1997; 94: 157-160
Article info
Publication history
Published online: November 23, 2017
Accepted:
September 22,
2017
Received in revised form:
September 19,
2017
Received:
July 6,
2017
Identification
Copyright
© 2017 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.
