April 2014

 

RESIDENTS

 

Academic grand rounds

Management of ocular cicatricial pemphigoid


by Maxwell Elia, MD, resident, and John J. Huang, MD, residency program director, Department of Ophthalmology and Visual Science, Yale University School of Medicine

 
 

John Huang, MD, residency program director, Department of Ophthalmology and Visual Science, Yale University School of Medicine

 

Figure 1. Right eye (OD): extensive symblepharon with temporal and nasal conjunctival adhesion to the inferior conjunctival fornix; left eye (OS): corneal pannus with ankyloblepharon of the upper lid and conjunctiva to the superior cornea and limbus

Figure 2. Right eye (OD): progression of the symblepharon and new ankyloblepharon of the temporal eyelid to the temporal cornea and limbus; left eye (OS): progression of the cornea pannus with keratinization of the corneal and conjunctival epithelium with extensive ankyloblepharon of the nasal and upper lid to the cornea Source (all): John Huang, MD

Case presentation

An 80-year-old woman was referred for bilateral eye pain. Her ocular history was significant for biopsy proven ocular cicatricial pemphigoid (OCP) for which she was receiving methotrexate 8 mg subcutaneous weekly injections. Aside from OCP, the patient was in good health. The patient previously underwent weekly epilations in the left eye, but she now complained of bilateral eye pain. On exam, she was 20/400 OD and counting fingers OS. Her IOP was normal and her extraocular movements were full. Anterior exam was significant for bilateral symblepharon and forniceal shortening as well as ankyloblepharon with corneal keratinization in the left eye (Figure 1). In an attempt to achieve disease control, the patient was started on prednisone 60 mg daily in addition to serum tears and topical prednisolone acetate 1% four times daily. One month later, the patient presented with worsening symblepharon, ankyloblepharon and keratinization of both eyes. Her left eye also demonstrated worsening left upper eyelid cicatricial entropion. Mycophenolate mofetil 500 mg twice daily and tacrolimus ointment twice daily were recommended, in addition to her methotrexate, prednisone, and topical therapy. By the next visit, the patient's pain had improved, and her vision stabilized at counting fingers OD and hand motions OS. She complained of debilitating vision loss and wanted to discuss her options for visual recovery. Her anterior exam was notable for bilateral stage IV OCP (forniceal shortening, symblepharon, and corneal keratinization) as well as cicatricial entropion of the left upper eyelid (Figure 2).

Discussion

Cicatricial pemphigoid (CP) is a devastating systemic disease resulting from the deposition of autoantibodies at the epithelial-subepithelial junction of mucous membranes. The eyes are involved in 70% of the cases of CP. The condition can cause chronic conjunctivitis, subepithelial conjunctival fibrosis, fornix foreshortening, symblepharon and ankyloblepharon formation, meibomian gland obstruction, trichiasis, and eventual lacrimal gland obstruction. Vision lost is due to corneal scarring and infection. The most important consideration after diagnosis is ensuring that aggressive systemic immunosuppression has been used to achieve disease control prior to considering any surgical intervention. Corticosteroids, dapsone, azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophosphamide, and intravenous immunoglobulin therapy have all demonstrated efficacy in arresting progression of OCP.1 Given this patient's age, it is important to use non-corticosteroid immunosuppressants to limit steroid-related side effects.

Visual rehabilitation for these patients requires consideration of the multiple challenges caused by OCP. Dry eye, cicatricial entropion, trichiasis, limbal stem cell deficiency, and deficiency of mucin-producing goblet cells all threaten the survival of any corneal graft or the type I Boston keratoprosthesis.

After the inflammation is controlled, surgical planning of the eyelid, anterior segment and cornea can be done based on the visual needs of the patient and prognosis after surgery. Initial steps involve the repair of the cicatricial entropion of the left upper eyelid followed by fornix reconstruction with amniotic membrane grafting, as described by Barabino.2 After fornix reconstruction, the stability of the ocular surface and limbal stem cells should be assessed prior to consideration of corneal grafting. Limbal stem cell deficiency, especially in a severe case as our patient, may present a significant challenge to graft survival. Persistent epithelial defects or poor wound healing may occur, which could result in graft failure. Limbal stem cells from living relatives, expanded ex vivo in culture, may offer hope for such patients in the future, but this therapy remains investigational.3

We commonly wait a minimum of three months between anterior segment reconstruction and any type of corneal transplantation or keratoprosthesis surgery. This will allow time for healing, resolution of inflammation from the surgery, and time to monitor for recurrence of symblepharon, which may recur in one-third of cases. Given that this patient has limited functional vision due to the significant eyelid and anterior segment involvement, we have recommended a type I or type II Boston keratoprosthesis, depending on the quality of the ocular surface after anterior segment reconstruction.

Outcome

This patient is scheduled to undergo combined repair of the left upper eyelid cicatricial entropion with lysis of symblepharon, ankyloblepharon and fornix reconstruction with amniotic membrane grafting. She will be further considered for placement of a keratoprosthesis.

References

1. Foster CS, Sainz De La Maza M. Ocular cicatricial pemphigoid review. Curr Opin Allergy Clin Immunol. 2004; 5:435-9.

2. Barabino S, Rolando M, Bentivoglio G, Mingari C, Zanardi S, Bellomo R, Calabria G. Role of amniotic membrane transplantation for conjunctival reconstruction in ocular-cicatricial pemphigoid. Ophthalmology. 2003; 110: 474-80.

3. Burman S, Sangwan V. Cultivated limbal stem cell transplantation for ocular surface reconstruction. Clin Ophthalmol. 2008; 2: 489-502.

Editors' note: Drs. Elia and Huang are affiliated with Yale University School of Medicine, Department of Ophthalmology and Visual Science, New Haven, Conn. They have no financial interests related to this article.

Contact information

Huang: john.huang@yale.edu

Management of ocular cicatricial pemphigoid Management of ocular cicatricial pemphigoid
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