Erosive Lichen Planus With Ocular Involvement: A Case Report

Introduction

Lichen planus (LP) is a chronic, idiopathic inflammatory disease mediated by T lymphocytes that can affect the skin, mucous membranes, nails, and hair follicles [1]. The prevalence is estimated at 0.5% to 2% of the general population; oral mucosal involvement is the most frequent manifestation [2]. Ocular involvement, although rare, is clinically significant because it may lead to chronic conjunctivitis, cicatricial changes, keratoconjunctivitis sicca, and – in severe cases – vision-threatening complications [3,4]. Among general practitioners and ophthalmologists unfamiliar with this presentation, ocular LP may be easily mistaken for other cicatrizing conjunctivitides such as ocular cicatricial pemphigoid, Stevens-Johnson syndrome, or chronic allergic conjunctivitis [5]. Pathogenesis involves an autoimmune reaction directed against basal keratinocytes, with deposition of immunoglobulins and complement at the basement membrane zone [6]. Histopathology and direct immunofluorescence (DIF) findings are important for diagnosis; in many real-world settings, images or slides may not be available.

Treatment of LP depends on the severity and affected sites. Topical corticosteroids are the first-line therapy for mucocutaneous disease, whereas systemic immunosuppressants (e.g., azathioprine, cyclosporine, mycophenolate mofetil, methotrexate) may be required for extensive or refractory disease [7,8]. In cases of ocular LP, tear substitutes, topical immunomodulators (such as cyclosporine A), and systemic therapy are often necessary to control inflammation and prevent scarring [9]. This case highlights the diagnostic challenges of ocular LP, the importance of interdisciplinary collaboration, and the need for long-term follow-up.

Case Report

A 69-year-old woman was referred for evaluation of bilateral ocular irritation, photophobia, and visual decline that began 3 months after uneventful cataract surgery. Her medical history was notable for hypertension and hypothyroidism. She denied use of topical medications beyond postoperative corticosteroids. She had no history of tobacco smoking, alcohol consumption, or substance abuse.

On examination, best-corrected visual acuity measurements were 20/150 in the right eye and 20/80 in the left eye. Slit-lamp examination revealed bilateral conjunctival hyperemia (+2), diffuse superficial punctate keratitis, and meibomian gland dysfunction. No symblepharon was observed. Intraocular pressure and fundoscopy findings were unremarkable. The initial impression was chronic ocular surface inflammation, possibly post-surgical or immune-mediated.

Further review revealed a 2-year history of painful oral ulcers, worsening dysphagia, 20-kg weight loss over 3 months, and nail brittleness. Physical examination identified violaceous plaques on the extremities and distal onycholysis. Intraoral lesions included reticulated white patches and erosions involving the buccal mucosa and tongue. Figure 1 shows the clinical appearance before treatment, including oral mucosa, cutaneous lesions on the upper limbs, slit-lamp examination with fluorescein, and nail changes.

Given the presence of clinically significant onychodystrophy, nail bed biopsy was performed on the right fourth fingernail. Histopathological analysis revealed superficial perivascular dermatitis exhibiting a lichenoid interface reaction pattern, consistent with LP-related changes. This finding supported a diagnosis of systemic erosive LP involving the nails.

Incisional oral biopsy findings initially were non-diagnostic. Repeat biopsy from the buccal mucosa showed a dense lichenoid lymphocytic infiltrate, basal cell degeneration, and apoptotic keratinocytes (Civatte bodies), consistent with lichenoid mucositis. Both DIF and indirect immunofluorescence (IIF) findings were negative. Despite these negative findings, the histology and clinical features supported a diagnosis of erosive LP. Hepatitis C serology results were negative. Conjunctival biopsy was not performed because mucosal lesions were available and the patient was reluctant.

Laboratory evaluation showed negative results concerning antinuclear antibodies, an extractable nuclear antigen panel, and enzyme-linked immunosorbent assay testing for bullous pemphigoid antigens BP180 and BP230, as well as desmoglein 1 and 3 antibodies. There was no evidence of paraneoplastic disease. The differential included mucous membrane pemphigoid, pemphigus vulgaris, paraneoplastic pemphigus, and Sjögren syndrome.

Due to extensive mucosal and ocular involvement, the case was discussed by a multidisciplinary team (ophthalmology, dermatology, rheumatology). Azathioprine (100 mg/day) was selected as a steroid-sparing agent based on the patient’s comorbidities and favorable safety profile. A 2-week course of oral corticosteroids (prednisone 0.5 mg/kg/day) was prescribed for rapid symptom control. No topical ocular immunosuppressants were used. Artificial tears and lid hygiene were maintained.

After 1 month, best-corrected visual acuity measurements improved to 20/40 in the right eye and 20/20 in the left eye. Conjunctival injection and keratitis subsided, oral pain diminished, and appetite improved. Figure 2 illustrates the same sites during immunosuppressive therapy with azathioprine. Four months later, there was substantial improvement in mucosal and cutaneous lesions, with partial resolution of onychodystrophy. The patient remained stable on azathioprine monotherapy with regular follow-up. Figure 3 shows clinically significant improvement after 4 months of treatment across all affected tissues.

After 12 months of follow-up, the disease remained stable without progression of conjunctival scarring and considerable relief of ocular discomfort. Oral lesions also improved; no malignant transformation was evident. The patient continues to attend interdisciplinary follow-up.

Discussion

Ocular involvement in LP is underrecognized but may be the initial sign of systemic disease. Manifestations include chronic conjunctival hyperemia, lid margin inflammation, punctate keratitis, symblepharon, and eventual forniceal foreshortening [2–5]. Due to similarities with mucous membrane pemphigoid and other cicatrizing disorders, LP often remains undiagnosed until irreversible fibrosis occurs [6].

Histologically, LP exhibits basal cell degeneration, a band-like lymphocytic infiltrate, and Civatte bodies. DIF results usually are positive for fibrinogen or complement at the basement membrane zone [7]. In the present case, both DIF and IIF results were negative, although this finding does not exclude LP. Therefore, joint consideration of histology and clinical observations is essential. Histopathological evidence of interface dermatitis on nail biopsy further supported a diagnosis of systemic LP. Nail involvement in LP may range from longitudinal ridging to severe dystrophy; histological confirmation is rarely performed but may aid in diagnosing ambiguous or overlapping presentations.

Although both DIF and IIF test results were negative in the present case, this finding does not rule out the diagnosis of LP. There is evidence that DIF may yield negative results in up to 30% of biopsy-proven oral LP cases, particularly when sampling is performed on older or non-ulcerated lesions [8]. The absence of linear deposition of fibrinogen or complement at the basement membrane zone does not exclude the diagnosis when typical histopathological features (e.g., band-like lymphocytic infiltrate, basal cell degeneration, Civatte bodies) are present. Negative IIF findings are expected in LP because circulating autoantibodies are not characteristic of the disease, unlike pemphigoid or pemphigus. In the present case, the constellation of clinical signs across multiple mucocutaneous sites, supported by histology, provided sufficient evidence to confirm erosive LP despite negative immunofluorescence findings. A limitation of this report is the absence of histopathology and DIF images – these studies were performed externally. Therefore, only clinical images of ocular, oral, cutaneous, and nail involvement are included as documentation of the disease course. A conjunctival biopsy could provide diagnostic confirmation but was deferred because adequate evidence was obtained from oral mucosa and due to patient preference. The potential Koebnerization triggered by cataract surgery is notable, given that trauma is a known precipitating factor for LP flares [4]. Similar timing between surgery and ocular symptoms has been described in other immune-mediated ocular diseases.

The decision to initiate azathioprine was based on the presence of severe multisite mucosal disease. Although other agents (e.g., methotrexate, cyclosporine) are available, azathioprine offers a favorable profile in older patients and allows steroid tapering [9,10]. Improvement within 30 days likely resulted from corticosteroids and supportive care, whereas sustained remission was achieved through azathioprine. Topical ocular treatments (e.g., cyclosporine or tacrolimus drops) were not used in this case but may benefit patients with predominant ocular symptoms. Future studies should investigate optimal ocular regimens in erosive LP. Notably, hepatitis C was excluded as a potential diagnosis because its association with LP is well documented [11] and relevant serology findings were negative.

Management of ocular LP remains challenging due to its rarity and the lack of standardized treatment guidelines. First-line therapy usually involves topical corticosteroids and lubricants. However, systemic immunosuppression is frequently required in cases with extensive mucosal involvement or ocular manifestations [12]. Azathioprine – used in the present case – is reportedly effective in controlling ocular inflammation and preventing scarring [13]. Other therapeutic options include cyclosporine A, mycophenolate mofetil, tacrolimus, and methotrexate [14,15]. Our patient remained stable after 1 year of follow-up, highlighting the importance of long-term monitoring. Malignant transformation of oral LP is a well-known complication, with an estimated risk of 0.5% to 2% [16]. Therefore, continued surveillance is crucial.

To our knowledge, this is one of few published reports to provide multimodal clinical documentation of erosive LP involving the skin, oral mucosa, nails, and ocular surface, along with a documented 12-month follow-up. This documentation strengthens the relevance of the case by demonstrating both the interdisciplinary diagnostic process and the long-term stability achieved with systemic immunosuppression.

Conclusions

Ocular surface inflammation may represent an early or underrecognized manifestation of systemic erosive LP. In patients with chronic conjunctivitis or keratitis refractory to conventional therapy – especially when accompanied by mucosal, cutaneous, or nail abnormalities – LP should be included in the differential diagnosis.

This case illustrates how ophthalmologic evaluation can serve as the initial step in identifying a multisite autoimmune condition. Early recognition and referral enabled a timely histopathologic diagnosis and appropriate systemic treatment. Interdisciplinary collaboration and the use of immunosuppressive therapy were essential to achieve clinical remission and prevent permanent ocular and mucosal sequelae.

This case adds to the limited body of literature on ocular LP by providing long-term, multimodal clinical documentation. A high index of suspicion, combined with anatomical pattern recognition and proactive systemic evaluation, can substantially improve outcomes among patients with erosive LP involving the eye.