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December 26, 2016 00:02

Pemphigoid scars .Causes.Symptoms .Diagnostics.Treatment

Synonyms: bullous dermatitis sinehialny atrophying slimy Lorta-Jacob, pemphigoid

benign mucosal pemphigoid causes and pathogenesis of the rumen is not fully understood.The pathogenesis is of great importance in the immune system breach, as obnaruzhayutsya blood circulating lgG-antibody and deposition of IgG and complement component-NW in the mucosal basal membrane skin.

Symptoms scarring pemphigoid.The disease is common in women after 50 years.The clinical picture includes dermatosis lesion mucous membrane of eyes, oral cavity, rare - nose, pharynx, urinary tract and skin.Approximately 40% of patients with scarring pemphigoid begins with the phenomenon of conjunctivitis, accompanied by photophobia and lacrimation.First, usually one eye is affected, with time (on average 3 to 6 months) and a second eye is involved.Gradually appear ephemeral subconjunctival subtle bubbles, which patients can not pay attention.Start of scar formation in the subconjunctival tissue clinically manifested in the form of sm

all adhesions between the lower and upper eyelids or the conjunctiva of eyelids and the eyeball.In the pathological process involved the entire conjunctiva.As a result of scarring observed shrinkage of the conjunctiva, conjunctival sac fusion (simblefaron), the eyelids are fused with the eyeball, eye gap narrows, there is limited mobility of the eyeball, developing ectropion with trihiazisom, deformation tear ducts, blurred and perforation of the cornea.The process can lead to blindness.

Approximately 30% of patients the disease begins with the defeat of the oral mucosa (often the soft palate, tonsils, cheeks and tongue), where apparently intact mucosa or eritromatoznom background bubbles appear with thick tire.Bubbles appear and then disappear, recurring for years in the same field.Bubble sizes of 0.3 to 1 cm or more with serous or hemorrhagic content.After the destruction of Tyre bubble formed maloboleznennye erosion, not prone to peripheral growth.Within a few months or years in the oral mucosa appear adhesions and scar-atrophic changes with impaired function of language.If it affects the nasal mucosa observed atrophic rhinitis with the formation of adhesions in the future between the nasal septum and shells.There may be spikes in the throat, in the corners of the mouth, esophagus structure, anus, urethra, phimosis, adhesions between the small labia and impaired function of these organs.

Rarely observed skin lesions.At the same time the bubbles on the skin, usually sporadic and rarely become generalized.The rash often located on the scalp, face, trunk, external genitalia, the area around the navel and anus, at least - of the limbs.They usually appear after the rash on the mucous membranes and in very rare cases, preceded by changes in the mucous membranes.Bubbles have different amounts (0.5 to 2 cm in diameter), a transparent liquid or hemorrhagic.After opening the bubbles formed pink, slightly moist erosion, which quickly covered with dry crusts.Erosion epiteliziruyutsya to form atrophic scars.Possible recurrence of dermatosis on the same field.

When scarring pemphigoid Nikolsky sign is negative, the lesions Tzanck cells are never found.The general condition of patients, as a rule, is not disturbed.

Histopathology.Histological examination of the skin and conjunctiva exhibit located subepithelial bubbles without acantholysis.The contents of the bladder marked eosinophils in the papillary layer of submucosal tissue - edema and significant infiltration consisting mainly of lymphocytes and histiocytes.In the later stages of the disease develop fibrosis and submucosal layer of the upper dermis.

differential diagnosis spend with normal and erythematous pemphigus vulgaris, bullous pemphigoid Lever, Stevens-Johnson syndrome, a form of bullous dermatitis herpetiformis, a disease Bskhcheta.

Treatment of pemphigoid rubtsuyuschegotakoe same as in bullous pemphigoid.Effective systemic glucocorticosteroids, DDS, a combination of corticosteroids with or presotsilolom delagilom.Applied vitamins A, B, E, biostimulants (aloe) and absorbable (lidasa) drugs, topically - glucocorticosteroids as aerosols and chipping.