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Cicatricial Pemphigoid - Causes, Symptoms, Diagnosis and Treatment

Published on Mar 24, 2022   -  4 min read

Abstract

Cicatricial pemphigoid is a rare autoimmune disorder that can affect the body's mucous membrane. Read the article below for more detail.

Contents

What Is Cicatricial Pemphigoid?

Cicatricial pemphigoid, also known as mucous membrane pemphigoid, is a chronic autoimmune disorder that results in blisters that predominantly affect the mucous membranes, including the skin and the conjunctiva. If only eyes are involved, it is referred to as ocular pemphigoid. It usually scars, hence if left untreated, this can even result in blindness when it affects the eyes. The risk of scarring depends on the location of disease activity.

What Is the Etiology for Cicatricial Pemphigoid?

The exact etiology is unknown, but according to the existing evidence, it supports a type 2 hypersensitivity response by our own body (autoantibody) against the epithelium of the basement membrane. Thus, the normal structure and function of the basement membrane are disrupted. This allows the epidermis to separate from the dermis, which manifests clinically as blisters and erosions. The oral cavity is the common site of involvement extending upto the pharynx posteriorly. There are severe cases that can affect mucous membranes anywhere in the body, such as sinuses, genitals, anus, and cornea.

What Are the Signs and Symptoms of Cicatricial Pemphigoid?

Eyes - Eye irritation, such as burning sensation or excessive tearing. The fornices (permits freedom of movement of the eyelids) are shortened, which causes adhesions between the eyelid and the eyeball. Later, when the disease progresses, they fuse. Severe scarring can result in blindness. The lesion is first present in one eye, which may progress to the other in a couple of years. There may be chronic tear deficiency when lacrimal glands (tear glands) are affected, which leads to dry eyes.

Clinically, the following are present:

Oral Cavity - There occurs recurrent painful erosions and desquamative (red, swollen ulcerative appearance of the gums) gingivitis where the lesions heal with scarring. Any mucosal site in the mouth may blister. When the pharynx is involved, it presents as hoarseness in voice or difficulty in swallowing. Progressive scarring may lead to esophageal stenosis (narrowing of the esophagus). Supraglottic (upper part of larynx including epiglottis) involvement may lead to airway obstruction requiring emergency treatment.

Nasal Involvement - Nasal bleeding (epistaxis), nasal crusting, and bleeding after blowing the nose are seen.

Skin - Vesicles that are small blisters or bulla that are bleeding large blisters are seen. They may heal with scarring. Itchiness may be present at the blister site, or generalized itchiness may be present. Common sites include the scalp, head and neck, hands, or trunk. Scalp involvement may lead to hair loss.

Genitals - On the genitalia, painful erosions involving the clitoris, labia, glans, or shaft of the penis may be seen.

How to Diagnose Cicatricial Pemphigoid?

The recommended tests to diagnose cicatricial pemphigoid includes:

  1. Biopsy of the skin lesion.

  2. Histopathological studies.

  3. Direct immunofluorescence (DIF).

They are performed on uninvolved skin or mucous membranes around the lesion. Histopathological studies reveal subepithelial blisters, infiltrates containing plasma cells, eosinophils, and neutrophils.

DIF demonstrates a linear band of antibodies (IgG) and precipitates complement (C3) in the basement membrane of the epithelia. Indirect immunofluorescence (IIF) can show linear deposition of IgA antibodies along with IgG and C3, suggestive of mucous membrane pemphigoid. A salt split technique is used in IIF that evaluates the presence of both IgG and IgA. Western blot, immunoprecipitation, and immunoelectron microscopy are also used. There is a positive Nikolsky sign (the upper layer of skin slips away from the lower layers when rubbed).

Imaging studies such as CT (computed tomography) scans and barium swallows may help evaluate the upper airway or the esophagus. Routine blood tests do not show any significant change from the normal range. Sometimes, there can be elevated immunoglobulins (antibodies), erythrocyte sedimentation rate (a test that measures how quickly the erythrocytes settle at the bottom of the test tube), and acute phase reactants (present during inflammation in the body).

What Is the Treatment for Cicatricial Pemphigoid?

What Are the Complications of Cicatricial Pemphigoid?

Conclusion:

A multidisciplinary approach is recommended in patients with cicatricial pemphigoid to provide adequate care. The disease is characterized by intermittent exacerbation and waning of disease activity. Due to the chronic nature of the disease and potentially severe complications, the patient should be counseled to remain compliant with the medical therapy and have regular follow-up visits.

Last reviewed at:
24 Mar 2022  -  4 min read

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