Scarring pemphigoid: causes, symptoms, diagnosis, treatment

Synonyms: bullous synechial atrophic mucous dermatitis of Lort-Jacob, benign pemphigoid of the mucous membranes

The causes and pathogenesis of cicatricial pemphigoid are not fully understood. In the pathogenesis, a disorder in the immune system is of great importance, since circulating IgG antibodies and deposition of IgG and the C3 component of complement in the area of the basement membrane of the skin of the mucous membrane are detected in the blood.

Symptoms of cicatricial pemphigoid. The disease often occurs in women over 50 years of age. The clinical picture of the dermatosis includes lesions of the mucous membrane of the eyes, oral cavity, rarely - nose, pharynx, urethra and skin. In approximately 40% of patients, cicatricial pemphigoid begins with conjunctivitis, accompanied by photophobia and lacrimation. At first, one eye is usually affected, with time (on average from 3 to 6 months) the second eye is involved. Gradually, ephemeral subconjunctival barely noticeable vesicles appear, which patients may not pay attention to. The onset of scar formation in the subconjunctival tissue is clinically manifested in the form of small adhesions between the lower and upper eyelids or the conjunctiva of the eyelids and the eyeball. The entire conjunctiva is involved in the pathological process. As a result of scarring, the conjunctiva shrinks, the conjunctival sac fuses (symblepharon), the eyelids fuse with the eyeball, the palpebral fissure narrows, the mobility of the eyeball is limited, ectropion with trichiasis, deformation of the lacrimal canals, clouding and perforation of the cornea develop. The process can lead to blindness.

In approximately 30% of patients, the disease begins with damage to the oral mucosa (often the soft palate, tonsils, cheeks, and uvula), where blisters with a thick cap appear on an apparently unchanged mucosa or on an erythromatous background. The blisters appear and disappear, recurring for years in the same places. The size of the blisters is from 0.3 to 1 cm or more with serous or hemorrhagic contents. After the destruction of the blister cap, painless erosions are formed that are not prone to peripheral growth. Over the course of several months or years, cicatricial adhesive and atrophic changes appear on the oral mucosa with impaired tongue function. When the nasal mucosa is damaged, atrophic rhinitis is observed with subsequent formation of adhesions between the nasal septum and turbinates. Adhesions may occur in the pharynx, corners of the mouth, structures of the esophagus, anus, urethra, phimosis, adhesions between the labia minora and dysfunction of these organs.

Skin lesions are rare. Skin blisters are usually isolated and rarely become generalized. Eruptions are often located on the scalp, face, torso, external genitalia, areas around the navel and anus, and less often on the extremities. They usually appear after rashes on the mucous membranes and in very rare cases precede changes in the mucous membranes. Blisters vary in size (from 0.5 to 2 cm in diameter), with clear or hemorrhagic fluid. After the blisters open, pink, slightly moist erosions form, which quickly become covered with dry crusts. Erosions epithelialize with the formation of atrophic scars. Recurrence of dermatosis in the same places is possible.

In cicatricial pemphigoid, Nikolsky's symptom is negative; Tzanck cells are never detected in the lesions. The general condition of patients is usually not affected.

Histopathology. Histological examination of the conjunctiva and skin reveals subepithelial vesicles without acantholysis. Eosinophils are found in the vesicle contents, and edema and significant infiltration, consisting mainly of lymphocytes and histiocytes, are observed in the papillary layer of the submucosal tissue. In the late stages of the disease, fibrosis of the submucosal layer and the upper part of the dermis develops.

Differential diagnosis is carried out with common and erythematous pemphigus, Lever's bullous pemphigoid, Stevens-Johnson syndrome, bullous form of herpetiform dermatitis, and Bschet's disease.

Treatment of cicatricial pemphigoid is the same as for bullous pemphigoid. Systemic glucocorticosteroids, DDS, a combination of glucocorticosteroids with presocilol or delagyl are effective. Vitamins A, B, E, biostimulants (aloe) and absorbable (lidase) drugs are used, locally - glucocorticosteroids in the form of aerosols and injections.

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