|Year : 2021 | Volume
| Issue : 4 | Page : 615-618
Isolated conjunctival lichen planus: A rare cause of cicatrizing conjunctivitis
Diego Paredes1, Arturo Grau1, Silviana Barroso1, Juan Manríquez2, Pablo Zoroquiain3
1 Department of Ophthalmology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile
2 Department of Dermatology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile
3 Department of Pathology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile
|Date of Submission||03-Dec-2020|
|Date of Acceptance||27-Apr-2021|
|Date of Web Publication||09-Oct-2021|
Dr. Pablo Zoroquiain
Marcoleta 377 Piso 7, Santiago Centro, Santiago 830000
Source of Support: None, Conflict of Interest: None
A 58-year-old female diabetic patient with keratinization of the gray line, areas of linear scar traction, and forniceal shortening was evaluated. Cicatrizing conjunctivitis was diagnosed. Conjunctival biopsy was performed and the histopathological study revealed a prominent band-like lymphocytic infiltrate. Direct immunofluorescence (DIF) showed a heavy fibrinogen deposition which was irregular in the basement membrane consistent with lichen planus (LP). A full dermatological evaluation was unremarkable. The final clinicopathologic diagnosis was isolated conjunctival lichen planus. As cicatrizing conjunctivitis may have different etiologies with differing treatment strategies, it is important to diagnose this entity and start treatment with anti-inflammatory drugs to avoid vision loss.
Keywords: Cicatrizing conjunctivitis, direct immunofluorescence, lichen planus
|How to cite this article:|
Paredes D, Grau A, Barroso S, Manríquez J, Zoroquiain P. Isolated conjunctival lichen planus: A rare cause of cicatrizing conjunctivitis. Indian J Ophthalmol Case Rep 2021;1:615-8
|How to cite this URL:|
Paredes D, Grau A, Barroso S, Manríquez J, Zoroquiain P. Isolated conjunctival lichen planus: A rare cause of cicatrizing conjunctivitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 28];1:615-8. Available from: https://www.ijoreports.in/text.asp?2021/1/4/615/327682
Cicatrizing conjunctivitis can be considered a true diagnostic challenge. Furthermore, diagnosis and treatment must be established early because chronic inflammation can produce severe and irreversible damage to the ocular surface causing a drastic decline in vision and quality of life.
Differential diagnosis includes autoimmune diseases such as ocular cicatrizing pemphigoid, linear immunoglobulin (Ig)A disease, graft versus host disease, Stevens–Johnson syndrome; infectious diseases such trachoma and fungal infections; inflammatory conditions such rosacea, allergic conjunctivitis, Sjögren syndrome, trauma, chemical and radiation damage, and neoplasms such sebaceous carcinoma. Herein, we present a case of isolated conjunctival lichen planus.
| Case Report|| |
A 58-year-old female diabetic patient consulted at the Cornea Department due to a 1-year history of bilateral tearing. She was evaluated previously in another center and allergic conjunctivitis was diagnosed, supported by a normal tear drainage test. Treatment with artificial tears, steroids, topical mast cell stabilizers, and oral antihistamines was indicated, but there was no improvement in the symptoms.
On current physical examination [Figure 1], her visual acuity was 0.95 and 1.0 with their respective correction. Schirmer's test without anesthesia was normal. Intraocular pressure was 10 mmHg in both eyes. On slit-lamp examination, keratinization of the gray line and agglutination of the eyelashes with flaking and collarettes were observed. In the lower tarsus, areas of linear scar traction were observed together with bilateral inferior fornices shortening. Minimal superficial epitheliopathy in the lower third of both corneas was seen. No relevant fundus findings were visualized.
|Figure 1: Clinical findings. (a) Hyperemic upper tarsus conjunctiva with subtle fibrosis (OD) (b) Upper tarsus linear fibrosis (OS) (c and d) Symblepharon with shortening of inferior fornix (c: OD, d: OS)|
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Blood tests were negative for anti-antineutrophil cytoplasmic antibodies (ANCA), anti-extractable nuclear antigen (ENA), Anti-Ro, Anti-La, and complement tests (C3 and C4), ruling out autoimmune causes of cicatrizing conjunctivitis. The working diagnosis was cicatrizing pemphigoid and a biopsy with direct immunofluorescence (DIF) was performed.
Biopsies of the bulbar conjunctiva from both the eyes (two samples for each eye) and oral mucosa were obtained without incidents. Pathologic examination revealed conjunctival mucosa with acanthosis, squamous metaplasia associated with a prominent band-like lymphocytic inflammatory infiltrate [Figure 2]a of the lamina propria with intraepithelial extension and interface phenomenon [Figure 2]b. DIF showed an irregular spiculate with a melting candle wax appearance and positivity for fibrin in the basement membrane [Figure 2]c. IgA, IgM, IgG, C3, and C5b were negative. The morphological and immunofluorescent findings were consistent with conjunctival lichen planus. A Full-body skin examination by a dermatologist did not reveal any evidence of cutaneous involvement.
|Figure 2: Morphological and immunofluorescence findings. (a) At low power, acanthosis of the epithelium and a band-like subepithelial infiltrate is observed (H and E, 10X). (b) At high power, the infiltrate is predominantly lymphocytic with intraepithelial extension and destruction of the epithelia-stromal junction *(H and E 40X). (c) Direct immunofluorescence demonstrates an irregular and duplicated deposition of fibrin in the base membrane (anti-fibrin antibody, FITC 40X)|
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The patient was treated with 0.1% topical cyclosporine twice a day, fluorometholone 0.1% three times a day and preservative-free artificial tears every 2–3 h and oral cyclosporine 5 mg/kg. After a 12-month follow-up, the patient is better with a normal Schirmer's test, static forniceal shortening, and a significant reduction of symptoms.
| Discussion|| |
Lichen planus (LP) is a rare autoimmune disease that affects the skin and mucous membranes. Its prevalence is unknown. However, some studies report an estimated prevalence between 0.07 and 0.84%. It usually affects middle-aged adults but there are some reports in the pediatric population. The etiology of LP is not entirely known, but it has been suggested that it might be the product of an immunological reaction mediated by T-cells against the mucosal and skin basement membranes. LP may present in two forms: cutaneous and mucosal. Cutaneous LP presents with skin lesions that are purple, polygonal, pruriginous, and papular. They usually start on the wrists, forearms, and trunk. Generally, the cutaneous form is a self-limiting disease that regresses in 2 years. Mucosal forms usually affect most frequently the oral mucosa and tongue, but may occur also in the anus, genitalia, and aerodigestive tract. Mucosal LP is a chronic condition that has no definitive treatment nor cure and usually presents as whitish reticular lesions.
Conjunctival involvement in patients with mucosal LP can be a significant cause of morbidity and potential irreversible vision loss. Generally, 8.3% of cicatrizing conjunctivitis not secondary to pemphigoid is due to LP. It usually presents as chronic cicatrizing keratoconjunctivitis in association with other mucosal involvement.
Isolated conjunctival LP is an extremely rare condition. It refers to LP that has exclusive conjunctival involvement. Only 11 cases of this condition have been reported [Table 1]. Most cases presented with irritative symptoms and cicatrizing conjunctival changes were the most common finding on ocular examination. Most of the patients received treatment with corticosteroids and cyclosporine, with a favorable subsequent evolution.
|Table 1: Case reports in literature of isolated conjunctival lichen planus|
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Diagnosis of LP relies on the clinical characteristics and the histopathological analysis. All the patients with cicatrizing conjunctivitis of unknown cause should be studied with conjunctival and oral mucosa to improve sensitivity. Furthermore, samples should be fixed in formalin for a morphologic evaluation and also sent in fresh using Michel's medium to maintain tissue immunogenicity for direct immunofluorescence. Classic histopathological findings of conjunctival LP are an interface inflammation with a characteristic DIF pattern: A fibrinogen irregular deposition with a candle wax appearance and duplication of the basement membrane. There are no signs of IgA or IgG deposition.
The treatment for ocular LP includes several strategies such as corticosteroid eye drops, topical cyclosporine, systemic immunosuppressants, and amniotic membrane transplantation for severe cases. There are no reports of comparison between the prognosis of conjunctival LP associated with systemic involvement and isolated conjunctival LP. Some authors suggest an increased awareness of this diagnosis to start early anti-inflammatory treatment to avoid irreversible visual loss.
| Conclusion|| |
LP is an infrequent conjunctival condition and can present as an isolated conjunctival form without skin or other mucosal involvement. Since the treatment of each etiology of cicatrizing conjunctivitis is different, a conjunctival biopsy with immunofluorescence is a useful tool to confirm the diagnosis and start a specific treatment.
We acknowledge Peter von Dassow for his contribution providing language help.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]