Summary about Disease
Cicatricial pemphigoid (CP), also known as mucous membrane pemphigoid (MMP), is a rare, chronic autoimmune blistering disease primarily affecting the mucous membranes of the body, such as the mouth, eyes, nose, throat, and genitals. It can also involve the skin. The disease is characterized by blisters that rupture and heal with scarring (cicatrization), which can lead to significant functional impairment, particularly in the eyes, potentially causing blindness.
Symptoms
Symptoms vary depending on the location of the affected mucous membranes and skin. Common symptoms include:
Mouth: Painful blisters and erosions, redness, difficulty eating or swallowing, scarring.
Eyes: Redness, burning, itching, tearing, blurred vision, sensitivity to light, scarring of the conjunctiva, potential blindness.
Nose: Crusting, bleeding, nasal obstruction, hoarseness.
Throat: Difficulty swallowing, hoarseness, scarring.
Genitals: Painful blisters and erosions, scarring.
Skin: Blisters that may be tense or flaccid, scarring.
Causes
Cicatricial pemphigoid is an autoimmune disease. This means the body's immune system mistakenly attacks healthy tissues. Specifically, in CP, the immune system produces antibodies that target proteins (e.g., BP180, BP230, laminin 332) in the basement membrane zone, the area that connects the epidermis (outer layer of skin) or epithelium (lining of mucous membranes) to the underlying connective tissue. This attack leads to inflammation and blister formation. The exact trigger for this autoimmune response is unknown in most cases. Some possible factors under investigation are:
Genetic predisposition
Environmental Triggers
Drug-induced
Medicine Used
Treatment aims to suppress the immune system and reduce inflammation. Common medications include:
Topical Corticosteroids: Creams or ointments applied directly to the affected areas to reduce inflammation.
Systemic Corticosteroids: Oral medications like prednisone to suppress the immune system.
Immunosuppressants: Medications like methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, rituximab, or dapsone to suppress the immune system.
Tetracycline Antibiotics: Sometimes used for their anti-inflammatory properties.
Intravenous Immunoglobulin (IVIG): Can be used in severe cases to modulate the immune system.
Is Communicable
No. Cicatricial pemphigoid is not communicable. It is an autoimmune disease, not an infection, and cannot be spread from person to person.
Precautions
Precautions focus on managing symptoms, preventing complications, and protecting affected areas:
Gentle Oral Hygiene: Use a soft toothbrush and avoid harsh mouthwashes if the mouth is affected.
Eye Care: Consult an ophthalmologist regularly and follow their recommendations for managing eye symptoms (e.g., artificial tears, lubricating ointments).
Sun Protection: Protect skin from sun exposure.
Avoid Irritants: Avoid foods or substances that irritate the affected areas.
Adherence to Treatment: Follow the prescribed medication regimen closely.
Regular Monitoring: Undergo regular check-ups with your healthcare provider to monitor the disease and treatment side effects.
How long does an outbreak last?
The duration of an "outbreak" or flare-up of cicatricial pemphigoid is highly variable and unpredictable. The disease is chronic and can persist for years or even a lifetime. Individual flare-ups can last for weeks to months. Even with treatment, periods of remission may be followed by periods of increased disease activity.
How is it diagnosed?
Diagnosis typically involves:
Clinical Examination: Evaluation of the characteristic blisters and erosions, especially in the mucous membranes.
Biopsy: A small sample of affected tissue is taken and examined under a microscope to look for specific features of CP, such as subepithelial blistering.
Direct Immunofluorescence (DIF): A specialized test performed on the biopsy sample to detect the presence of antibodies (IgG, IgA, and/or complement C3) deposited in the basement membrane zone.
Indirect Immunofluorescence (IIF): Blood tests to detect circulating antibodies that react with the basement membrane zone. This test is less sensitive than DIF.
ELISA (Enzyme-Linked Immunosorbent Assay): Blood tests to identify specific antigens (e.g., BP180, BP230, laminin 332) that the antibodies are targeting.
Timeline of Symptoms
The timeline of symptoms varies greatly among individuals. However, a general progression might look like this:
Initial Stage: Subtle symptoms such as redness, burning, or itching in the affected areas, often followed by the appearance of small blisters.
Active Blistering Stage: Blisters form and rupture, leading to erosions and ulcers. This phase can last for weeks to months.
Healing and Scarring Stage: As the blisters and erosions heal, scarring (cicatrization) occurs. This scarring can lead to significant functional impairment, especially in the eyes and mouth.
Chronic Stage: The disease becomes chronic, with periods of remission (reduced disease activity) and exacerbations (flare-ups). Scarring progresses over time.
Important Considerations
Early Diagnosis and Treatment: Early intervention is crucial to minimize scarring and prevent complications, especially blindness.
Multidisciplinary Approach: Management often requires a team of specialists, including dermatologists, ophthalmologists, dentists, and otolaryngologists (ENT doctors).
Potential for Blindness: Ocular involvement is a major concern, and regular ophthalmological monitoring is essential.
Impact on Quality of Life: CP can significantly impact quality of life due to pain, functional impairment, and psychological distress. Support groups and counseling can be helpful.
Treatment Side Effects: Immunosuppressive medications can have significant side effects, so regular monitoring is necessary.