Mucous membrane pemphigoid is a rare chronic dermatosis from the group of pemphigoids, characterized by a lesion of the mucous membranes without involvement in the pathological process of the skin. The first vesicular elements appear on the mucous membrane of the oral cavity, upper respiratory tract, genitals. Later, the mucous membrane of the eyes is affected. The bubbles are tense, filled with serous contents, bursting, leaving behind deep erosions with an outcome in an atrophic scar. The bulls are fixed. The disease is diagnosed cytologically (absence of acantholytic cells) and histologically (subepithelial bladder). Treatment is symptomatic, taking into account concomitant pathology.
Mucous membrane pemphigoid (scarring pemphigoid) is a non-cytolytic benign dermatosis that resolves by scarring. The disease has a gender coloring: mostly elderly women are ill. The pemphigoid of mucous membranes is not characterized by seasonality, endemic distribution. Since the cause of the disease and the exact mechanism of its development are not completely clear, there is no consensus among dermatologists about whether dermatosis is an independent disease or a type of bullous pemphigoid.
A.L. Mashkillayson described the mucosal pemphigoid with confirmation of the process’s goodness in 1959. The disease has adverse consequences for the quality of life, as it affects the mucous membrane of the eyes, conjunctiva, scarring of which causes atrophy of the membranes of the ocular apparatus. Chronic course of pemphigoid with frequent relapses can lead to blindness (in 30-60% of cases). The faster the development of the process, the worse the prognosis, taking into account the scars on the mucous membranes of the upper respiratory tract, complicating normal breathing, scars on the esophagus, leading to the impossibility of normal food intake, cachexia. Usually negative changes are diagnosed after 3 years of pathology. Cases with malignancy of scars are described.
The etiology of the disease is unknown, the autoimmune process is taken as the basis for the mechanism of development. Under the influence of “provocateurs” (UVI, thermal burn, prolonged drug therapy), the antigen-antibody reaction mechanism is triggered in the skin, where the own tissues of the epidermis act as the antigen. A distinctive feature of the pathology is the production of antibodies in the patient’s body not to a single antigen (cells of the basal layer of the epidermis), but to a whole complex of antigens located in different layers of the mucous membranes. The bubbles are located subepithelially, delaminate more than one layer of cells; erosions at their opening are deep, resolved not by epithelization, but by an atrophic scar. It is believed that the antigenic complex is localized on the mucous membranes, since it normally plays an important role in the healing of their impaired integrity.
Initially, in 90% of cases, the oral mucosa is affected. Serous bulls spontaneously appear, which quickly open up, leaving behind painful erosions. Resolution occurs through scarring, while the localization of blisters is extremely important: bullae on the frenulum practically immobilize the tongue, blisters on the tonsils, cheek mucosa, soft palate – complicate the process of swallowing, normal access of food to the body, resulting in dystrophic syndrome, cachexia. The mucous membranes of the genitals and upper respiratory tract are involved in the process, the defeat of which occurs according to the same scenario.
The mucous membranes of the eyes are affected independently. The process is initially one-sided, with the passage of time the second eye is affected. Visually noticeable are the symptoms of catarrhal inflammation of the conjunctiva with a rash of small transparent bubbles that quickly open, scar, form adhesions, depriving the patient of the opportunity to close the eyelid. All subsequent relapses add corneal ulceration, new scars with different localization. There is scarring of the eyelids, atrophy of the tear ducts with drying of the mucous membranes, narrowing of the eye slit, static eyeball, blindness. The pathological process, which has existed for many years, is characterized by a specific clinical picture: the patient has eyes on his face that resemble the eyes of sculptures, the cornea acquires a cloudy whitish hue, practically not passing light through itself.
The clinic of the disease is typical. Nikolsky’s symptom is negative. Despite this, laboratory research methods play a crucial role in diagnosis. Cytologically, acantholytic cells are not detected. The histological picture is typical: the formation of a vesicular subepithelial element without signs of acantholysis. Examination of the biopsy using direct and indirect immunofluorescence confirms the presence of antigenic complexes in the epidermis, states the presence of specific antibodies. Electron microscopy is very rarely used.
Differentiate mucous membrane pemphigoid with true pemphigoid, erosive lichen erythematosus, Behcet’s disease, lupus erythematosus, bullous pemphigoid, Stevens-Jones syndrome, During dermatitis, dystrophic epidermolysis bullosa, aphthous dermatitis.
A patient with a lesion of the mucous membranes is subject to treatment, both by a dermatologist and an ophthalmologist. Treatment is symptomatic. The therapy is based on the use of sulfones (dapsone), glucocorticoids (prednisolone), immunosuppressants (azathioprine). In severe cases, a combination of corticosteroids with cytostatics is possible. The consequences of scarring are treated promptly. In case of eye damage, intraocular injections of hormonal drugs, special contact lenses are prescribed. Externally, hormonal ointments, gels, aerosols are used. The adhesive process according to indications (after consultation with a plastic surgeon and an immunologist) is eliminated by tissue transplantation. The prognosis is relatively unfavorable, since the quality of life of patients is significantly impaired, depends on the timeliness of diagnosis and treatment.