Ophthalmologic Manifestations of Cicatricial Pemphigoid Clinical Presentation
- Author: C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor: Hampton Roy Sr, MD more...
History
- Ocular symptoms
- Red eye
- Tearing
- Dry eye
- Blepharospasm
- Itching
- Grittiness
- Heavy eyelid
- Foreign body sensation
- Decreased vision
- Burn sensation
- Photophobia
- Diplopia
- Other symptoms
- Skin lesions
- Oral lesions
- Esophageal lesions
- Pharyngeal lesions
- Laryngeal lesions
- Anal lesions
- Tracheal lesions
- Vaginal lesions
- Urethral lesions
Physical
- External examination
- Conjunctival hyperemia (red eye)
- Quiet eye
- Entropion
- Skin lesions
- Nasal lesions
- Mouth lesions
- Discharge (ie, catarrhal, mucous, membranous)
- Slit lamp examination
- Eyelids
- Trichiasis
- Distichiasis
- Meibomian gland dysfunction
- Blepharitis
- Conjunctiva
- Papillae
- Follicles
- Keratinization
- Subepithelial fibrosis
- Conjunctival shrinkage
- Foreshortening of fornices
- Symblepharon
- Ankyloblepharon
- Cornea
- Superficial punctate keratitis
- Epithelial defect
- Stromal ulcer
- Neovascularization
- Keratinization
- Limbitis
- Conjunctivalization
- Stromal opacity
- Perforation
- Eyelids
- Based on clinical findings, progression of disease can be divided into 4 stages.
- Stage I is characterized by chronic conjunctivitis with mild conjunctival and/or corneal epitheliopathy with subepithelial conjunctiva fibrosis, best seen at the tarsal conjunctiva as fine, white striae.
- Stage II is characterized by cicatrization with conjunctival shrinkage, distorted anatomy, and foreshortening of fornices, as shown below.
Ocular cicatricial pemphigoid, stage II. Note the fornix foreshortening. - Stage III is characterized by the presence of symblepharon (see image below). Subepithelial scarring alters the orientation of lashes, causing aberrant lash growth. In addition, cicatricial entropion may occur.
Ocular cicatricial pemphigoid, stage III. Note the symblepharon. - Stage IV is the end stage, consisting of a dry eye with keratinization of the cornea and ankyloblepharon, which immobilizes the globe (see first image below). Profound keratopathy can develop secondary to eyelid disorders, tear insufficiency, and corneal exposure. Corneal epitheliopathy, persistent epithelial defects, stromal ulceration, and neovascularization may be present (see second image below). The cornea may become completely scarred, vascularized, and keratinized.
Ocular cicatricial pemphigoid, stage IV. Note the ankyloblepharon and ocular surface keratinization.
Corneal neovascularization with ulceration and stromal thinning after persistent epithelial defect in a patient with ocular cicatricial pemphigoid.
Causes
The cause of OCP is unknown. Genetic factors and several triggers can increase the likelihood of the onset of OCP.
- Individuals with HLA-DR2, HLA-DR4 (HLA-DR*0401), and HLA-DQw7 (DQB1*0301) are more susceptible to OCP.
- In some patients, systemic practolol therapy and topical antiglaucoma drugs (eg, pilocarpine, timolol, epinephrine, Humorsol, idoxuridine, phospholine iodide) have been identified as triggers of OCP.
Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84:527-663. [Medline].
Chan RY, Bhol K, Tesavibul N, et al. The role of antibody to human beta4 integrin in conjunctival basement membrane separation: possible in vitro model for ocular cicatricial pemphigoid. Invest Ophthalmol Vis Sci. Sep 1999;40(10):2283-90. [Medline].
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. Winter 1996;36(1):41-60. [Medline].
Cordero Coma M, Yilmaz T, Foster CS. Tumour necrosis factor-alpha in conjunctivae affected by ocular cicatricial pemphigoid. Acta Ophthalmol Scand. Nov 2007;85(7):753-5. [Medline].
Power WJ, Neves RA, Rodriguez A, et al. Increasing the diagnostic yield of conjunctival biopsy in patients with suspected ocular cicatricial pemphigoid. Ophthalmology. Aug 1995;102(8):1158-63. [Medline].
Hall VC, Liesegang TJ, Kostick DA, et al. Ocular mucous membrane pemphigoid and ocular pemphigus vulgaris treated topically with tacrolimus ointment. Arch Dermatol. Aug 2003;139(8):1083-4. [Medline].
Foster CS, Wilson LA, Ekins MB. Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology. Apr 1982;89(4):340-53. [Medline].
Foster CS, Ahmed AR. Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study. Ophthalmology. Nov 1999;106(11):2136-43. [Medline].
Sami N, Letko E, Androudi S, et al. Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up. Ophthalmology. Jul 2004;111(7):1380-2. [Medline].
Foster CS, Chang PY, Ahmed AR. Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology. May 2010;117(5):861-9. [Medline].
Daoud Y, Amin KG, Mohan K, Ahmed AR. Cost of intravenous immunoglobulin therapy versus conventional immunosuppressive therapy in patients with mucous membrane pemphigoid: a preliminary study. Ann Pharmacother. Dec 2005;39(12):2003-8. [Medline].
Heiligenhaus A, Shore JW, Rubin PA, et al. Long-term results of mucous membrane grafting in ocular cicatricial pemphigoid. Implications for patient selection and surgical considerations. Ophthalmology. Sep 1993;100(9):1283-8. [Medline].
Sainz de la Maza M, Tauber J, Foster CS. Cataract surgery in ocular cicatricial pemphigoid. Ophthalmology. Apr 1988;95(4):481-6. [Medline].
Neumann R, Tauber J, Foster CS. Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid. Ophthalmology. Jun 1991;98(6):858-62. [Medline].
Foster CS, Neumann R, Tauber J. Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol. 1992;82(3):223-9. [Medline].
Saw VP, Dart JK, Rauz S, et al. Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes. Ophthalmology. Feb 2008;115(2):253-261.e1. [Medline].

