Cicatricial Pemphigoid

Key Points
Autoimmune disorder generally affecting mucous membranes
Condition is due to the production of antibodies which attack the mucous membranes of the body
Consists of blistering lesions, usually affecting the mouth or eyes

Cicatricial pemphigoid is an autoimmune disorder which affects the mucous membranes of the body. While it most often affects the inside of the mouth and the eyes and eyelids, it can appear in the nostrils, esophageal track, trachea, and genitals. It can also affect the face, neck and scalp in some cases.

The condition consists of blistering lesions in the affected area, and can be accompanied by attendant symptoms depending on what area is affected. Lesions on the surface of the eye or underside of the eyelid can be accompanied by conjunctivitis and a gritty sensation accompanied by pain. Lesions may heal and leave scar tissue, which can lead to an impairment of vision or even blindness. Lesions in the mouth initially form along the gumline, but may spread to the tongue, palate, lips and floor of the mouth. Lesions may be extremely painful. Lesions in the nostrils may crust, causing discomfort, and bleeding may occur when blowing the nose. On the genitals, lesions can form on the clitoris and labia in women, on the shaft of the penis in men, and in the perianal area in both. In 25-30% of affected persons, lesions may also form on the skin, causing itching and bleeding under trauma. Lesions forming in the esophageal track are very dangerous and could be life-threatening.
Cicatricial Pemphigoid is caused by the body producing antibodies which direct the immune system to attack the mucous membranes, causing the blistering lesions. While it is almost exclusively seen in elderly persons, it can in rare cases affect children. Males are twice as likely to develop the condition.

Differential Diagnosis (Other conditions with similar appearance)

Erythema multiforme
Bullous pemphigoid
Linear IgA dermatosis
Drug-induced bullous disorders
Pemphigus vulgaris
Epidermolysis bullosa
Paraneoplastic pemphigus
Epidermolysis bullosa acquisita

Diagnosis
Key Points
Initial diagnosis based on skin appearance
Skin Biopsy and other testing will be performed to confirm diagnosis and to rule out other, similar appearing conditions
In cases where the esophageal track is affected, imaging tests may be performed to evaluate severity

Cicatricial pemphigoid is initially diagnosed by appearance of the lesions. Further testing, including skin Biopsy, will be performed to confirm the diagnosis and to rule out other, similar appearing conditions. In cases where the esophageal track is affected, imaging tests such as CT scans may be performed.

Treatment
Goal of treatment is to prevent further formation of lesions, and to assist the healing process and prevent scarring
Treatment is dictated by the parts of the body involved
*Common treatments include topical, injection, and oral steroids, and immunosuppressants

Cicatricial pemphigoid can be very difficult to treat. In many cases it is chronic and progressive, and remissions are rare. In cases where the mouth, nose and skin are affected, common treatments include Topical steroids, intralesional steroid Injections, and short courses of orally-administered corticosteroids in severe cases. In cases where the eye is affected, aggressive intervention is usually managed by ophthalmology to prevent irreversible scarring of the eye.

It should be noted that proper care of lesions is very important. Promoting healing, minimizing scarring, and avoiding factors which may worsen the condition are the best ways to combat permanent damage.