Scar pemphigoid: causes, symptoms, diagnosis, treatment

Cicatricial pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with corneal opacification. Early symptoms are hyperemia, discomfort, itching, and discharge; progression leads to damage to both eyelids and the cornea and sometimes blindness. Diagnosis is confirmed by biopsy. Treatment may require systemic immunosuppressive therapy.

Cicatricial pemphigoid has the following synonyms: benign pemphigoid of the mucous membrane; ocular cicatricial pemphigoid).

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What causes cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune disease in which inflammation of the conjunctiva results from the formation of antibodies to the basilar membrane. The disease is not associated with bullous pemphigus.

Symptoms of cicatricial pemphigoid

Typically beginning as chronic conjunctivitis, the disease progresses to symblepharon (fusion of the conjunctiva of the eyelids to the globe); trichiasis (abnormal growth of eyelashes); "dry" keratoconjunctivitis; neovascularization, opacification, and keratinization of the cornea; and wrinkling and keratinization of the conjunctiva. Chronic corneal epithelial defects may lead to secondary bacterial ulceration, scarring, and blindness. Involvement of the oral mucosa with ulceration and scarring is common, and cutaneous involvement characterized by scarring bullae and erythematous macules is rare.

Cicatricial pemphigoid is characterized by gradual development of non-specific symptoms of irritation, burning, and lacrimation, making it easy to miss the disease. Papillary conjunctivitis is associated with diffuse conjunctival hyperemia. Subconjunctival bullae may form and perforate, leading to ulcers and pseudomembranes. Subepithelial fibrosis, conjunctival wrinkling, and flattening of the contour of the folds and papillae.

The usually progressive course of the disease may be interrupted by episodes of subacute activity characterized by diffuse conjunctival hyperemia and edema.

Complications of cicatricial pemphigoid

• Dry eye is caused by a combination of destruction of the goblet cells and accessory lacrimal glands, as well as occlusion of the main lacrimal duct and meibomian orifice.
• Symblepharon is a serious complication in which adhesions form between the palpebral and bulbar conjunctiva. To better see it during the disease, it is necessary to pull the lower eyelid down and ask the patient to look up.
• Ankyloblepharon is characterized by the formation of adhesions in the outer corners of the eye slit between the upper and lower eyelids.
• Secondary keratopathy, which may be considered threatening, is caused by a combination of entropion with scarring of the palpebral conjunctiva, abnormal eyelash growth, secondary lagophthalmos due to symblepharon, dryness, and decreased limbal stem cells.

The terminal stage of the disease is characterized by keratinization of the corneal surface, obliteration of the fornices, corneal vascularization and ulceration, which may lead to secondary bacterial infection.

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Diagnosis of cicatricial pemphigoid

The diagnosis is made clinically by the presence of symblepharon without a history of local irradiation or severe, long-standing allergic conjunctivitis. It is confirmed by conjunctival biopsy demonstrating antibody deposits in the basilar membrane.

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Treatment of cicatricial pemphigoid

Tear substitutes and cryoepilation or electrolysis of abnormally growing eyelashes can improve the patient's condition and reduce the risk of eye infection. Systemic immunosuppression with dapsone and cyclophosphamide is indicated if scarring or corneal opacity progresses.

Local treatment of cicatricial pemphigoid: steroids are used in the acute stage;

• Tear substitutes are used to replenish the deficiency of one's own tears;
• Antibiotics are used after taking cultures from the conjunctiva and eyelids.

Subconjunctival injections of mitomycin C may be effective in preventing the progression of conjunctival scarring.

Silicone contact lenses are used with caution to protect the cornea from eyelash damage and drying. Hard scleral contact lenses can be effective in retaining the tear film on the corneal surface and protecting it from discharge and mechanical damage from the eyelids, but they do not prevent fornix scarring.

In most cases, systemic treatment of cicatricial pemphigoid is required:

• steroids are used for acute manifestations;
• Dapsone can be used in moderate cases to reduce tissue involvement;
• Cytotoxic agents (methotrexate, cyclophosphamide) may be useful in suppressing conjunctival inflammation and preventing progression of mucosal wrinkling. Azathiofrine is less effective as monotherapy but may be valuable in combination with other partially effective agents;
• Intravenous administration of immunoglobulins can be effective in persistent processes.

Surgical treatment of cicatricial pemphigoid is required in the following complications:

• Cicatricial inversion of the eyelid and abnormal growth of eyelashes.
• A severe manifestation of "dry" eye, when occlusion of the lacrimal puncta is necessary if they are not closed by scars.
• Large, widespread corneal defects may require tarsorrhaphy or injection of Chl. botulinum toxin into the levator to induce ptosis and promote healing.
• Keratoprosthetics can be used on eyes with progressive keratinization of the ocular surface.