Ocular Cicatricial Pemphigoid (OCP) Clinical Presentation

Updated: Jul 16, 2019
  • Author: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

The history of ocular cicatricial pemphigoid (OCP) is as follows:

  • 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

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Physical

Physical examination of ocular cicatricial pemphigoid (OCP) is as follows:

  • 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

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 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 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 Ocular cicatricial pemphigoid, stage IV. Note the ankyloblepharon and ocular surface keratinization.
Corneal neovascularization with ulceration and str Corneal neovascularization with ulceration and stromal thinning after persistent epithelial defect in a patient with ocular cicatricial pemphigoid.
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Causes

The cause of ocular cicatricial pemphigoid (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.

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Complications

Ocular complications of ocular cicatricial pemphigoid (OCP) include the following:

  • Corneal epithelial defects

  • Corneal stromal ulcers

  • Corneal perforation

  • Endophthalmitis

  • Glaucoma

Two types of lesions can occur. The most common lesion is a vesiculobullous eruption, similar to that of bullous pemphigoid. These lesions rupture spontaneously and heal without significant scarring. The second type of lesion is an erythematous localized plaque that evolves into recurring bullae, which can rupture and leave scars (ie, Brunsting-Perry dermatitis). Patients with OCP may present with skin lesions and lesions on other mucous membranes (eg, nose, mouth, esophagus, pharynx, larynx, urethra, vagina, anus).

Mouth involvement is the most common. Scarring of mucosa in the nose and the mouth can be debilitating. Nasopharyngeal involvement can manifest as ruptured vesicles of the nasal mucosa along with discharge, crusting, and epistaxis.

Patients with tracheolaryngeal lesions may present with hoarseness, dyspnea, and laryngeal stenosis. Aphonia can occur secondary to vocal cord involvement. Laryngeal stenosis and tracheal scarring with mucous accumulation may lead to fatal asphyxiation.

Progressive desquamative gingivitis typically results in bone loss and dental extraction.

Pharyngeal scarring can cause painful swallowing with subsequent malnutrition and weight loss. Patients suspected of having OCP must be questioned for the presence of dysphagia and difficulties in breathing. Endoscopic evaluation is required, if there is a possibility of tracheal or esophageal involvement.

Pain on defecation or rectal bleeding can manifest as a result of rectal involvement; however, esophageal involvement, typically is silent. Although the progressive scarring associated with esophageal inflammation results in esophageal strictures, which may produce dysphagia, even to the point of choking with attempted swallowing of food. As a consequence of this condition, death from asphyxiation is reported and is a major threat.

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