Cicatricial pemphigoid is a rare autoimmune disorder that can affect the body's mucous membrane. Read the article below for more detail.
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.
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.
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:
Conjunctivitis (inflammation of the conjunctiva of the eye).
Keratinization (filled with keratin) of corneal epithelium.
Shortening of the corneal sulcus.
Entropion (eyelid rolled inward that irritates the eye caused by the lashes).
Symblepharon (bulbar and palpebral conjunctiva form an abnormal adhesion to one another).
Long-term ankyloblepharon (adhesion of the edges of the upper eyelid with the lower eyelid).
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.
The recommended tests to diagnose cicatricial pemphigoid includes:
Biopsy of the skin lesion.
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).
In case of mild disease of the oral mucosa and skin, topical therapies can be effective. If the topical treatments are not effective, Dapsone (medicine to treat skin diseases) and systemic corticosteroids can be used. In addition, the importance of oral care has also been emphasized.
Proper eye care is essential. For mild to moderate eye lesions, only systemic corticosteroids or corticosteroids combined with Dapsone may be considered. Since dry eyes are familiar, lubricants must be applied as artificial tear drops or petroleum jelly. Excess fluid from the eyes must be cleaned to prevent secondary bacterial infections.
For severe or rapidly progressive cases, a combination of systemic corticosteroids and immunosuppressive agents is recommended. The recommended drugs are Azathioprine, Mycophenolate mofetil, and Cyclophosphamide.
In patients refractory to other therapies, high-dose intravenous immunoglobulin has been used.
Oral mucosal lesions result in painful scarring that can cause limitation in movement. Gingival lesions can result in loss of gingival tissue, loss of alveolar bone and teeth.
Ocular complications include decreased tear and mucin production, secondary infection, symblepharon, ankyloblepharon, corneal ulcers, and blindness.
Nasal complications include discharge, epistaxis, crust formation, chronic sinusitis, scarring, and impaired airflow.
Pharyngeal complications include hoarseness or loss of voice, supraglottic stenosis, and airway compromise.
Esophageal complications include difficulty swallowing, aspiration, and narrowing the air passage or food passage in the body (stricture formation).
Anogenital complications also include painful ulcerations, stenosis, and stricture formation.
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
Most Popular Articles
Do you have a question on Cicatricial Pemphigoid or ?Ask a Doctor Online