cicatricial pemphigoid

Introduction

Introduction to scar-like pemphigus Scar pemphigoid is mainly characterized by blisters, which occurs in the oral mucosa of the oral cavity and the eye-binding membrane, also known as benign mucosal pemphigus. The course of the disease is slow, with an average of 3 to 5 years, and some can be extended for a lifetime. Serious eye damage can affect vision and even blindness. Middle-aged and older women are more common, twice as many as men. basic knowledge The proportion of illness: 0.003% Susceptible people: more common in middle-aged and older women Mode of infection: non-infectious Complications: corneal ulcer

Cause

Causes of scar-like pemphigoid

Autoimmune disease.

Prevention

Scar pemphigoid prevention

Scar pemphigoid is more common, also known as benign mucosa pemphigus. The main manifestation of blisters is that the blister collapses to form an ulcer surface. Occasionally in the mouth, combined with the membrane. Severe eye problems can affect or even lose sight. Occurs in women, middle-aged and middle-aged people are more common, fewer deaths, slower course, there are reports that it is related to malignant tumors, such as rectal cancer, uterine cancer. The solution is treated with a solution of a corticosteroid preparation, and an anti-inflammatory and analgesic gargle is used. Patients with severe conditions are considered to use corticosteroids. Eliminate the effects of cross-reactive foreign antigens or eliminate the effects of various factors affecting the changes of autoantigens, such as preventing and treating infections, and avoiding the use of certain drugs that are prone to induce autoimmune reactions.

Complication

Scar pemphigoid complications Complications corneal ulcer

Scar pemphigoid usually begins with chronic conjunctivitis, progresses to sacral adhesions (sacral conjunctiva and eyeball scars), trichiasis (inward lashes), dry keratitis, corneal neovascularization and opacity and horns Or even blindness, when it involves the oral mucosa, ulcers and scars often form, such as throat scar formation, vulva, perianal, esophageal mucosa can also be affected. However, the characteristics of scars and erythema that are involved in the skin are uncommon. Due to eating difficulties, malnutrition and cachexia may occur, and the incidence of blindness is approximately 20% to 60%. There are reports of carcinogenesis on the scar of the oral mucosa.

Symptom

Symptoms of scar-like pemphigus Common symptoms Corneal ulcers Conjunctival edema and corneal ulcers The blister or bullae that grows in the ear hole damages the size of the cornea... Scars are scarred after injury

1. Oral: The earliest and most common is the damage of the gums, which is exfoliative gingivitis-like damage. The gingival margin and the attached erythema are diffuse erythema, and the blister is visible on the gingival sac. After the blister breaks, the red smooth ulcer surface remains, and the Nissl sign is negative. If the damage occurs in the uvula, soft palate, tonsil and pharynx, dysphagia, pain, scarring after healing, and adhesion to adjacent tissues may occur, causing deformity. If the lesion occurs in the mouth area, it may result in limited mouth opening or small mouth deformity.

2. Eyes: 50 to 85% of patients may have eye symptoms. Early is simple combination of membranous inflammation, local itching. Repeated episodes may occur with sputum-ball adhesion, varus varus, trichiasis, corneal damage, and the like. Corneal scars can cause vision loss.

3. Other mucous membranes: mucous membranes in the pharynx, trachea, esophagus, genitals, anus, etc. are occasionally involved, and narrow esophage can cause difficulty in swallowing.

4. Skin damage: The face and scalp are often involved, and the chest, abdomen, armpits, and limbs may also appear. The skin lesions showed ocular blisters on erythema or normal skin, Nissl's sign was negative, and the blister wall was thick and not easily broken.

Examine

Examination of scar pemphigoid

1. Pemphigus: Most of the mouth is the erosion surface left after the blister breaks, and the Nissl sign is positive. Lesions often occur in the chest, trunk and scalp, neck, armpits, groin and other vulnerable parts of the friction, which is a loose bullous, thin wall, Nissl positive. Pathological manifestations: acanthosis, epithelial bleb. Direct immunofluorescence: an antibody against cell-adhesive substances between layers of spine cells.

2. Bullous pemphigoid: thick wall tension bullae can be seen on the skin, Nissl's sign is negative. Pathological manifestations: no acanthosis, epithelial blister. Direct immunofluorescence: IgG and C3 were observed linearly in the basement membrane. Generally, the systemic symptoms are mild, the course of disease is long, and the prognosis is good.

3. Polymorphic erythema: acute onset, related to allergies. A large amount of blister in the mouth is smashed and the target erythema is visible on the skin.

4. Erosive lichen planus: The gums can be exfoliated sputum-like changes, the color is bright red, but the mucosa in other parts of the mouth can have white streaks.

Diagnosis

Diagnosis and differentiation of scar-like pemphigus

1. The gums are congested with obvious exfoliative gingivitis-like changes.

2. Scarring often occurs in soft palate and pharynx.

3. -ball adhesion.

4. Histopathological examination: epithelial blister, no acanthosis release.

5. Direct immunofluorescence: The basement membrane area has a continuous elongated fluorescent band.

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