Scarring pemphigoid: causes, symptoms, diagnosis, treatment

Synonyms: dermatitis bullous synechial atrophy mucous Lort-Jacob, pemphigoid benign mucous membranes

The causes and pathogenesis of the pemphigoid scarring to the end have not been studied. In pathogenesis, a violation in the immunity system is of great importance, since circulating lgG antibodies and deposition of the IgG and C3 complement components in the basal membrane of the mucosal skin are found in the blood.

Symptoms of pemphigoid scarring. The disease is often found in women after 50 years. The clinical picture of dermatosis includes damage to the eye mucosa, mouth cavity, rarely - nose, pharynx, urethra and skin. Approximately in 40% of patients scarring pemphigoid begins with the phenomenon of 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 also involved. Gradually, ephemeral subconjunctival, low-noticeable vesicles appear, to which patients may not pay attention. The beginning of scar formation in the subconjunctival tissue is clinically manifested in the form of small adhesions between the lower and upper eyelids or conjunctiva of the eyelids and the eyeball. In the pathological process, the entire conjunctiva is involved. As a result of scarring, wrinkling of the conjunctiva, fusion of the conjunctival sac (simblepharon), eyelids fuse with the eyeball, eye gap narrow, there is a restriction of mobility of the eyeball, ectropion with trichiasis develops, lacrimal canal deformation, opacity and perforation of the cornea. The process can lead to blindness.

Approximately 30% of patients with the disease begins with a lesion of the oral mucosa (often the soft palate, tonsils, cheeks and tongue), where on externally unchanged mucous membrane or on erythromatous background appear blisters with a thick cover. Bubbles then appear, then disappear, recurring for years in the same places. Bubble sizes from 0.3 to 1 cm or more with serous or hemorrhagic contents. After the destruction of the bladder cover, there are little painful erosions, not prone to peripheral growth. For several months or years on the mucous membrane of the mouth there are cicatrical-adhesive and atrophic changes with a violation of the function of the tongue. When the mucous membrane of the nose is affected, atrophic rhinitis is observed, with further formation of fusion between the nasal septum and the shells. There may be spikes in the pharynx, in the corners of the mouth, the structure of the esophagus, the anus, the urethra, phimosis, adhesions between the labia minora and the disruption of the function of these organs.

Skin lesions are rare. In this case, the blisters on the skin, as a rule, are single and rarely become generalized. Eruptions are often located on the skin of the scalp, face, trunk, external genitalia, areas around the navel and anus, less often - the limbs. Usually they appear after rashes on the mucous membranes and in very rare cases precede changes on the mucous membranes. Bubbles have a different value (from 0.5 to 2 cm in diameter), a transparent or hemorrhagic fluid. After opening the blisters, pink, slightly damp erosions are formed, which are quickly covered with dry crusts. Erosion is epithelialized with the formation of atrophic scars. Possible recurrence of dermatitis in the same places.

With cicatricial pemphigoid, Nikolsky's symptom is negative, Tzanck cells are never found in lesions. The general condition of patients, as a rule, is not violated.

Histopathology. In the histological examination of the conjunctiva and skin, subepithelial blisters are found, without acantholysis. In the contents of the bladder, eosinophils are noted, in the papillary layer of submucosal tissue - edema and significant infiltration, consisting mainly of lymphocytes and histiocytes. In the later stages of the disease fibrosis of the submucosal layer and the upper part of the dermis develops.

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

Treatment of the pemphigoid scarring is the same as in the bullous pemphigoid. Systemic glucocorticosteroids, DDS, a combination of glucocorticosteroids with presocilol or delagil are effective. Apply vitamins A, B, E, biostimulants (aloe) and absorbable (lidaz) drugs, topically - glucocorticosteroids in the form of aerosols and obkalyvaniya.

[1], [2], [3], [4], [5], [6], [7]