Updated Guidelines on Presentation, Diagnosis, and Treatment of Ocular Involvement of Lichenoid Dermatoses

Ocular manifestations of lichenoid dermatoses, such as lichen planus (LP), lichen planus pigmentosus (LPP), and lichen planopilaris (LPL), are rare and often overlooked by dermatologists, but lead to serious complications. An up-to-date review for dermatologists on ocular manifestations of LP, LPP and LPL, as well as guidelines on when to refer patients to the ophthalmologist, was published in Dermatologic Therapy.

• Lichen planus (LP)
  • Overview. LP is a chronic inflammatory mucocutaneous disease that predominantly affects the skin, followed by the oral and genital mucosa, as well as the esophagus, scalp, and nails. LP is most common in adult women between the 3^rd and 6^th decade of life. Diagnosis is confirmed via skin biopsy, which reveals a triad of band-like infiltrates, irregular acanthosis, and vacuolation of basal cells.
  • Ocular manifestations. This is rare and usually occurs with oral or cutaneous manifestations. Extraocular disease usually precedes eye involvement.
    • The most common presentation is conjunctivitis, followed by corneal involvement (keratitis), and punctal and/or canalicular lacrimal drainage obstruction.
      • Recently, a rare case of scleritis was reported.
    • Patients initially report blurred vision, redness, dry eyes, mild mucous discharge, and foreign body sensation. Later in the disease, patients report severe vision loss, refractory dry eye disease (DED), and ocular pain.
    • The most frequent ocular finding is conjunctival subepithelial fibrosis in the inferior and/or superior palpebral conjunctiva in the context of mild DED. These lesions appear as white conjunctival streaks in the palpebral conjunctiva and may go unnoticed without lid eversion. If left untreated, these lesions can lead to conjunctival scarring with symblepharon formation. Symblepharon leads to entropion with or without trichiasis.
    • Late-stage disease is characterized by corneal opacity, neovascularization, thinning, and ulceration. Recently, a case of corneal perforation was reported.

• Lichen planus pigmentosus (LPP)
  • Overview. LPP is an idiopathic inflammatory condition characterized by brown macules on sun-exposed and flexural areas of the skin; it most often occurs in those with Fitzpatrick skin types III, IV, and V Sometimes a burning sensation or mild pruritis may occur with the lesions. The forehead and preauricular region of the face are most often affected. Comorbidities associated with LPP include hypothyroidism, chronic urticaria, hepatitis C, and vitiligo. Biopsy is required to confirm diagnosis since LPP mimics other pigmentary disorders. Histopathological findings include epidermal thinning, focal vacuole degeneration of the basal cell layer, interface infiltration of lymphocytes, and pigmentary incontinence in the dermis.
  • Ocular manifestations. The most common manifestation is “racoon eyes” –periorbital and eyelid hyperpigmentation, typically hyperpigmented linear dark brown macules in the lines of Blaschko, below the inferior eyelid and the lateral canthal area. The macules may appear on the upper eyelid; there is only 1 case reported that involved other ocular structures.

• Lichen planopilaris (LPL)
  • Overview. LPL is a rare disease, mainly affecting the scalp, and  is divided into 3 clinical presentations: the classic form, frontal fibrosing alopecia (FFA; Kossard disease), and Graham-Little-Piccardi-Lasseur syndrome. The classic form presents with multifocal areas of hair loss that involve scalp, eyebrows, axillae, or pubis hairlines with perifollicular erythema and scale, with or without scalp pruritis. FFA presents with frontal hairline recession that is evident when contrasting the forehead sun-damaged skin or when using the solitary hair sign. LPL mainly affects postmenopausal women with Fitzpartick skin types I, II, and III, but may rarely affect children. Conjunctival tissue analysis reveals loss of mucin-producer goblet cells, which are replaced with keratinocytes, multiple necrotic keratinocytes, and a linear inflammatory infiltrate along the mucosal surface.
  • Ocular manifestations. Ocular manifestations of LPL include eyelash loss, and conjunctival and corneal scarring. A study reported statistically significant altered values of tear film break up time (TFBUT), ocular surface staining parameters, and meibomian gland function compared to healthy control patients. There was 1 case reported in a young girl of undiagnosed LPL presenting with photophobia, red eyes, tearing, and eye pain.

• Natural history of LP, LPP and LPL in the eye
  • Plaques in LP and periorbital hyperpigmentation in LPL. Innocuous and do not progress to severe disease.
  • Ocular surface (OS) involvement. Usually begin at the conjunctiva with conjunctival intraepithelial inflammation, resulting in destruction of mucin-producer goblet cells, which leads to inflammatory tear production and hyperosmolar tissue damage. The tissue damage results in further epithelial and goblet cell loss in the conjunctiva and decreased OS wettability. Chronic inflammation causes OS keratinization, which presents clinically as mild subepithelial fibrosis (white streaks) of the palpebral conjunctiva.
  • Eyelashes and eyelids. A third of patients present with blepharitis and meibomian gland dysfunction, which impair the lipid layer production of the tear film and contribute to dry eyes. Patients with LPL have presented with absent sebaceous glands.
  • Scarring. This leads to lacrimal obstruction with punctum stenosis, producing epiphora, secretion, and an increased exposure of inflammatory tears to the OS. If left untreated, subepithelial fibrosis leads to fornix foreshortening, symblepharon formation, and OS keratinization with or without ankyloblepharon.
  • End-stage disease. Characterized by corneal involvement. Corneal microtrauma from tarsal fibrosis and chronic inflammation of the entire OS leads to overexpression of matrix metalloproteinases and proinflammatory cytokine. This causes epithelial breakdown without epithelial healing, which leads to corneal scarring (observed as an opacified “white eye”), neovascularization, stromal melting, ulceration, and an increased risk for perforation.

• Diagnosis of ocular involvement: dermatologist vs ophthalmologist
  • A dermatologist can perform an initial assessment to detect common initial signs and symptoms, including a thorough ophthalmic history. Patients usually report a history of fluctuating dry eye and blurred vision. The dermatologist can use the Ocular Surface Disease Index (OSDI), a validated 12-item questionnaire, to further assess these symptoms, as well as tear production using the Schirmer test I and entropion, trichiasis, conjunctival hyperemia, lacrimal gland swelling, madarosis, and gross corneal opacities or leucomas with a bare eye exam of the OS.
    • Any abnormalities require a referral to an ophthalmologist.
  • Assess eyelashes for meibomian gland dysfunction by looking for inflammation, hyperemia, and telangiectasias in the eyelid margin. Dermatologists can perform eyelid eversion to look for conjunctival scarring and symblepharon formation.
    • If found, the patient requires a referral to an ophthalmologist.
  • Perform a thorough evaluation of the skin, skin appendages, and mucosal surfaces since eye and skin manifestations usually coexist.
  • Consider side effects of certain drugs, such as hydroxychloroquine given for LPL.
  • To assess subtle changes in the OS, patients require a slit-lamp evaluation by an ophthalmologist.

• Treatment
  • Patients with confirmed or suspected extraocular LP, LPP or LPL and dry eye symptoms. After distinguishing between true ocular involvement and DED, prescribe topical lubricants for lid-hygiene as well as preservative-free artificial tears. If the patient is unresponsive to this treatment, refer to the ophthalmologist.
  • First-line treatment for mild conjunctival or corneal involvement of LP. Corticosteroid and cyclosporine (CsA) eyedrops, alone or in combination. Prescribe topical corticosteroids or tacrolimus ointment for eyelid pruritis. Intense lubrication with artificial tears or ophthalmic ointments is crucial at every stage of the disease.
    • Successful management with fluorometholone 0.1% qid and CsA 2% bid has been reported.
  • Patients unresponsive to topical therapy. Systemic corticosteroids and immunosuppressive therapy such as azathioprine (AZT), cyclosporine (CsA), methotrexate (MTX), dapsone, mofetil mycophenolate (MMF), and cyclophosphamide (CYC). If long-term treatment is necessary, treat the patient with adjunctive vitamin D and calcium. Perform necessary clinical and laboratory assessments to monitor for complications of long-term systemic corticosteroid treatment.
    • Local radiotherapy is no longer considered as an alternative therapy.
  • Ocular LP vs ocular mucous membrane pemphigoid (OMMP)
    • Ocular LP. Responds to topical steroids at initial stages.
    • OMMP. Always requires systemic immunosuppression, preferably with prednisone and CYC. Biologic agents (ie, rituximab) may be used instead of CYC for ovarian protection.
  • Surgical management of the OS. Amniotic membrane transplantation and corneal transplant is required when there is corneal ulceration or perforation. Lacrimal obstruction at any level may necessitate dacryocystorhinostomy.

Reference

Ruiz-Lozano RE, Hernández-Camarena JC, Valdez-Garcia JE, et al. Ocular involvement and complications of lichen planus, lichen planus pigmentosus, and lichen planopilaris: A comprehensive review. Dermatol Ther. Published online Sep 19, 2021. doi:10.1111/dth.15137