What Is a Cicatricial Pemphigoid?
Cicatricial pemphigoid is an autoimmune disease that causes inflammation due to the combination of anti-conjunctival basement membrane antibodies and antigens. Chronic, bilateral, progressive scarring and contraction of the conjunctiva with corneal opacity is a rare chronic blistering disorder of the mucosal tissue.
- English name
- benign mucosal pemphigoid
- Visiting department
- dermatology
- Multiple groups
- Elderly people, with an average age of onset of 58 to 65 years, and twice as many women as men
- Common locations
- Eye and oral mucosa
- Common symptoms
- Blepharoplasty, trichiasis, dry keratitis
Basic Information
Causes of cicatricial pemphigoid
- The disease is a subtype of bullous pemphigoid. Most patients have linear deposition of immunoglobulin G (IgG) and C3 in the epidermal basement membrane band.
Clinical manifestations of cicatricial pemphigoid
- Occurs in the elderly, with an average age of onset of 58 to 65 years. Female patients are twice as likely as men. They mainly invade the eye and oral mucosa. Nasal cavity, throat, esophagus, urethral opening, vagina, and anal mucosa are also affected. It usually starts with chronic conjunctivitis and progresses to blepharoplasty (adhesion of the eyelid and eyeballs), trichiasis (inward eyelashes), dry keratitis, corneal neovascularization and opacity and keratosis, or even blindness When the oral mucosa is involved, ulcers and scars are often formed, such as scar formation in the throat, vulva, perianal, and esophageal mucosa. But the characteristics of scarring and erythema when the skin is involved are uncommon.
Examination of cicatricial pemphigoid
- Laboratory inspection
- Direct immunofluorescence showed that the epidermal basement membrane had IgG and complement C3 and was linearly deposited.
- 2. Histopathological examination
- It is a subcutaneous blister. There is no slackness in the epidermis. There are lymphocytes, plasma cells, and eosinophils in the dermis, and the superficial fibrosis of the dermis is obvious in the later stage.
Diagnosis of cicatricial pemphigoid
- Diagnosis can be made based on the cause, clinical manifestations, and laboratory tests.
Scar Pemphigoid Treatment
- Treat the eyes with a solution of a corticosteroid preparation and use an anti-inflammatory and analgesic gargle. Consider glucocorticoids in severe cases. Tear fluid substitutes and frozen or electrolytic trichiasis make patients comfortable and reduce the risk of eye infections. Systemic immunosuppression is required for progressive scarring or corneal opacity, and dapsone or cyclophosphamide can be used. During treatment, the hematopoietic system, kidney and other organ functions should be monitored, and the drug should be used rationally under the guidance of experienced experts.