What causes Cicatricial pemphigoid?

What causes Cicatricial pemphigoid?

The exact cause of mucous membrane pemphigoid is unknown. MMP is an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses (antibodies or immunoglobulins) against “foreign” or invading organisms (antigens) begin to attack healthy tissue for unknown reasons.

What is Cicatricial pemphigoid?

Cicatricial pemphigoid is a subepidermal blistering disease that involves the mucous membranes and the skin. The oral cavity and the eye are most frequently involved. The clinical course is of long duration, and often there is significant scarring that can have devastating sequelae.

Can ocular cicatricial pemphigoid be cured?

There is no cure for pemphigoid but with proper long-term treatment and careful follow up it is usually possible to control the inflammation and the vision threatening side effects.

How is Cicatricial pemphigoid diagnosed?

Investigations

  1. Histology, direct immunofluorescence (DIF) and indirect immunofluorescence (IDIF) are used to diagnose CP. However, differentiation from similar blistering diseases (particularly bullous pemphigoid) is on clinical grounds.
  2. Imaging may be required to investigate the upper airway and oesophagus.

How long does pemphigoid last?

Bullous pemphigoid often goes away on its own in a few months, but may take as many as five years to resolve. Treatment usually helps heal the blisters and ease any itching.

What is pemphigoid in the eye?

Ocular mucous membrane pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with opacification of the cornea. Early symptoms are hyperemia and irritation; progression leads to eyelid and corneal damage and sometimes blindness. Diagnosis is sometimes confirmed by biopsy.

How common is ocular cicatricial pemphigoid?

The incidence of ocular cicatricial pemphigoid (OCP) is estimated at between 1 in 8,000 and 1 in 46,000 ophthalmic patients.

Is ocular cicatricial pemphigoid an autoimmune disease?

Ocular cicatricial pemphigoid, also known as mucous membrane pemphigoid (MMP), is an autoimmune disease that can cause scarring in the conjunctiva of your eyes. The conjunctiva is the inside lining of your eyes that keeps a smooth surface when you blink or close your eyes.

Can pemphigoid affect the eyes?

Ocular mucous membrane pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with opacification of the cornea. Early symptoms are hyperemia and irritation; progression leads to eyelid and corneal damage and sometimes blindness.

How can you tell the difference between pemphigus and pemphigoid?

Pemphigus is characterized by shallow ulcers or fragile blisters that break open quickly. Pemphigoid presents with stronger or “tense” blisters that don’t open easily. Those with pemphigoid are also more likely to have hot, red and itchy hive spots.

What happens if pemphigoid is left untreated?

The most common sites affected are the mouth and eyes. The rash and blistering may begin in one of these areas and spread to the others if left untreated. If it’s left untreated in the eyes, it may cause scarring, which in turn may lead to blindness.