Ocular Cicatrical Pemphigoid

Ocular cicatricial pemphigoid (OCP) is a rare form of mucous membrane pemphigoid (MMP) that affects the conjunctiva, which is the thin, transparent membrane that covers the white part of the eye, also known as the sclera, and lines the eyelids.1 This progressive autoimmune disease affects the mucous membranes throughout the body, resulting in ulceration and scarring.2

OCP usually appears in the sixth to eighth decade of life, and rarely in individuals younger than 30.3,4 The incidence has been reported to range from between 1 in 12,000 to 1 in 60,0005 and women are affected more than men by a ratio of 2 to 1.6,7

Approximately 61% of patients with cicatricial pemphigoid have eye involvement.8 In the early stages, these patients develop recurrent conjunctivitis, with associated tearing, irritation, burning, and occasional mucus drainage. Early treatment is critical since untreated, this disease progresses in up to 75% of patients.9,10,11 Without treatment, OCP can lead to keratinization of the ocular surface12 and dramatic scarring of the conjunctiva.13

What Causes OCP?

The exact underlying cause for OCP is unknown, but like other immune conditions, OCP results when the body overproduces antibodies that attack itself. It is possible for an environmental insult or a drug to trigger an immune attack on mucosal tissue in genetically predisposed individuals. However, identifying a precise cause remains challenging.

How Is OCP Diagnosed?

Diagnosing OCP can be challenging since the clinical course is variable. Sometimes, it starts out slowly and goes into remission, while other cases have rapid onset. In addition, other conditions, such as trauma and chemical burns and infection, can mimic OCP and make diagnosis even more difficult.

A thorough history is helpful since it often reveals that other systems, such as the gums, mouth or throat, have been affected. For example, the presence of mouth ulcers, which are often present in early disease, can help identify OCP. This knowledge, combined with inflammation or scarring of the conjunctiva, can help guide diagnosis, which can then be confirmed by conjunctival biopsy.

Treatment for OCP

Eye drops are not enough to treat OCP. While topical therapy can be used as an adjunct for surface disease, it should not be used in place of systemic therapy. Because OCP is a systemic immune disease, it must be managed with systemic immunosuppressant therapy. Without this, scarring will progress and can lead to permanent vision loss.

While receiving systemic therapy, patients with OCP need to see an eye doctor regularly to control conjunctival inflammation, prevent conjunctival and corneal scarring, and protect the ocular surface. Artificial tears, steroids, antibiotics, punctual plugs and cyclosporine may be prescribed.14

Following systemic treatment, which often lasts several years, remission is possible.

Scleral Lenses for OCP

Ocular surface protection is an important goal when treating OCP. However, drops are not usually sufficient to lubricate and protect the ocular surface for these patients. In many cases, a scleral lens should be considered, as these lenses can aid in the visual rehabilitation and ocular surface protection in patients with OCP.15

Scleral lenses are large-diameter contact lenses that vault over the entire corneal surface and rest on the white part of the eye, known as the sclera. Liquid fills the space between the eye and the back surface of the scleral lens. This liquid bandage protects the cornea from the ongoing mechanical shearing effect of the eyelids that occurs when you blink. It also continuously bathes the sensitive corneal tissue in OCP patients. Such protection can be beneficial in these cases and has been shown to help in both patient comfort and in maintaining the ocular surface.16 In addition, for people with OCP who also need vision correction, the fluid layer helps smooth out defects, providing a more uniform refractive surface.

If you have OCP, visit your eye doctor to determine the most appropriate management strategy for you.

 


[1] Arafat SN, Suelves AM, Spurr-Michaud S, et al. Neutrophil Collagenase, Gelatinase and Myeloperoxidase in Tears of Stevens-Johnson Syndrome and Ocular Cicatricial Pemphigoid Patients. Ophthalmology. 2014;121(1):79-87.

[2] Ophthalmic Pathology and Intraocular Tumors. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, 2014; pp54-56.

[3] External Disease and Cornea. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, 2014; pp344-345.

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[9] External Disease and Cornea. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, 2014; pp344-345.

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[11] Elder MJ, Bernauer W, Leonard J, Dart JK. Progression of disease in ocular cicatricial pemphigoid. Br J Ophthalmol. 1996;80(4):292-296.

[12] Zakka LR, Reche P, Ahmed AR. Role of MHC Class II Genes in the pathogenesis of pemphigoid. Autoimmunity Reviews. 2011;(11):40–47.

[13] Ward M. et al. Ocular Cicatricial Pemphigoid: atypical presentation as pseudopterygium and limbal stem cell deficiency. Eyerounds.org. Sep 2010.

[14] Neff AG, Turner M, Mutasim DF. Treatment strategies in mucous membrane pemphigoid. Ther Clin Risk Manag. 2008;4(3):617-626.

[15] Schornack MM, Baratz KH. Ocular cicatricial pemphigoid: the role of scleral lenses in disease management. Cornea. 2009 Dec;28(10):1170-2.

[16] Rosenthal P, and Cotter J. The Boston Scleral Lens in the management of severe ocular surface disease.Ophthalmology Clinics of North America. Mar 2003. 16(1):89-93.