Cicatricial pemphigoid: causes, symptoms, diagnosis, treatment

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

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

[1], [2], [3], [4], [5], [6]

What causes cicatricial pemphigoid?

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

Symptoms of cicatricial pemphigoid

Usually, starting as a chronic conjunctivitis, the disease progresses to the symphobaron (fusion of the eyelid conjunctiva with the eyeball); trichiasis (abnormal growth of eyelashes); "Dry" keratoconjunctivitis; neovascularization, opacification and corneal corneal and wrinkling and keratinization of the conjunctiva. Chronic epithelial defects in the cornea can lead to secondary bacterial ulceration, scarring and blindness. Often there is involvement of the mucous membranes of the mouth with ulceration and scarring, rarely involving skin, characterized by scarring of blisters and erythematous spots.

Cicatricial pemphigoid is manifested by gradual development of nonspecific symptoms in the form of irritation, burning and lacrimation, which makes it easy to miss the disease. Papillary conjunctivitis is associated with diffuse hyperemia of the conjunctiva. Subconjunctival blisters can be formed and perforated, which leads to the formation of ulcers and pseudomembranes. Subepithelial fibrosis, conjunctival puckering and flattening of the contour of folds and papillae.

Usually the progressing course of the disease can be interrupted by episodes of subacute activity characterized by diffuse conjunctival hyperemia and edema.

Complications of cicatricial pemphigoid

• The "dry" eye is caused by a combination of destruction of goblet cells and additional lacrimal glands, as well as the occlusion of the main lacrimal canal and the opening of the meibomian gland.
• Simblefaron is a serious complication, during which adhesions are formed between the palpebral and bulbar conjunctiva. To better see him in the process of illness, you need to pull the lower eyelid down and ask the patient to look up.
• Ankyloblopharone is characterized by the formation of adhesions in the outer corners of the eye gap between the upper and lower eyelids.
• Secondary keratopathy, which can be considered as threatening, is caused by a combination of eyelid twisting with scarring of palpebral conjunctiva, abnormal eyelash growth, secondary lagophthalmia due to simbelfarone, dryness and a decrease in the number of limbal stem cells.

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

[7], [8], [9], [10], [11], [12]

Diagnosis of cicatricial pemphigoid

The diagnosis is established clinically by the presence of simbelfarone without the presence in the history of local irradiation or severe long-term allergic conjunctivitis. It is confirmed by a biopsy of the conjunctiva, which shows the deposition of antibodies in the main membrane.

[13], [14], [15]

Treatment of cicatricial pemphigoid

Improve the condition of the patient and reduce the risk of infection of the eyes can replace tears and cryoepilation or electrolysis of improperly growing eyelashes. With the progression of scarring or clouding of the cornea, systemic immunosuppression with the use of dapsone and cyclophosphamide is indicated.

Local treatment of cicatricial pemphigoid steroids is used in acute stages;

• replacements of tears are used to fill the deficit of one's own tears;
• antibiotics are used after taking the crops from the conjunctiva and the eyelids.

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

Silicone contact lenses are used with caution to protect the cornea from damage caused by eyelashes and to dry out. Solid scleral contact lenses can be effective in keeping the tear film on the surface of the cornea and protecting it from exudates and mechanical damage for centuries, but they do not prevent scarring of the vaults.

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

• Steroids are used for acute manifestations;
• Dapsone can be used with moderate manifestations to reduce the involvement of tissues in the process;
• cytotoxic agents (methotrexate, cyclophosphamide) may be useful in suppressing conjunctival inflammation and preventing the progression of mucosal shrinkage. Azathioprine is less effective in monotherapy, but can be valuable when combined with other partially effective agents;
• The intravenous administration of immunoglobulins can be effective in a persistent process.

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

• Scarring eyelid and wrong growth of eyelashes.
• A severe manifestation of a "dry" eye, when occlusal tear points are necessary, if they are not covered by scars.
• Large common corneal defects may require tarzoraphias or injections of ChI toxin. Botulinum in the levator to stimulate ptosis and accelerate healing.
• Keratoprosthesis can be used on the eyes with progressive keratinization of the eye surface.