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Cicatricial Pemphigoid - Causes, Symptoms, Diagnosis and Treatment

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

Written by

Dr. Sowmiya D

Medically reviewed by

Dr. Kumari Monalisa

Published At March 24, 2022
Reviewed AtJanuary 22, 2024

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:

  • 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.

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?

  • 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.

What Are the Complications of Cicatricial Pemphigoid?

  • 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.

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.

Frequently Asked Questions

1.

Is Pemphigoid Curable?

Pemphigoid cannot be permanently cured, but certain medications can relieve the symptoms and ease discomfort. Treatment prevents the condition from getting worse. The sores and blisters reduce and sometimes can resolve completely. Bullous type of pemphigoid may clear by itself but can take around five years to resolve completely. Popping blisters with infection can cause sepsis (overactive response to infection), which can be life-threatening.

2.

Can a Blood Test Detect Pemphigoid?

Blood tests can be useful in identifying the antibodies associated with pemphigoid since it is an autoimmune (the body’s immune system detects self-tissues as foreign and attacks them) disorder. IIF and ELISA are common blood tests. IIF detects circulating antibodies and their pattern of deposition, while the ELISA test identifies and semi-quantifies antibodies to antigens present in bullous pemphigoid and other variants.

3.

What Medications Trigger Pemphigoid?

Sulfur-containing drugs, especially Furosemide, can trigger drug-induced pemphigoid. Other causative drugs include Amoxicillin, Ampicillin, Penicillin, Phenacetin, Penicillamine, Captopril, Tiopronin, beta-blockers, and Psoralen-ultraviolet-A light. Over 50 agents are considered responsible for causing drug-induced bullous pemphigoid, which includes diuretics (a drug type that increases the urine output), ACE inhibitors (drugs to treat hypertension), and antibiotics. 

4.

Can One Become Blind From Pemphigoid?

Ocular cicatricial pemphigoid is a type of mucous membrane pemphigoid (a less common, autoimmune disorder that affects the mucous membranes of the body, particularly the mouth and the eyes), which affects the eye, and if left untreated, can cause blindness. Studies have shown that about 25 to 30 percent of patients can turn blind due to the mechanism of the condition and due to scarring or clouding of the cornea (the transparent membrane that permits light to pass through). 

5.

Which Parts of the Oral Cavity Are Affected by Cicatricial Pemphigoid?

When the rare, autoimmune cicatricial pemphigoid disorder affects the mucous membranes, then it is called mucous membrane pemphigoid. The mouth is commonly affected by cicatricial pemphigoid. Common sites in the oral cavity include all areas like buccal mucosa (the inner lining of the cheeks), gingiva (gums), tongue, vermilion lips (the reddish or pink margin of the lip), and the palate (the roof portion of the mouth in the inside).

6.

What Type of Disease Is Pemphigoid?

Normally, the immune system of the body fights against harmful microorganisms like bacteria and viruses by producing antibodies. Pemphigoid is a type of autoimmune disease in which the immune system considers cells on the upper layer of the skin (epidermis) and mucous membranes as foreign substances and attacks them. When antibodies are produced against the protein-binding skin cells, the protein bonds break, skin becomes fragile, and fluid gets collected, forming blisters.

7.

How Long Does It Take for a Pemphigoid to Go Away?

Bullous type of pemphigoid can resolve on its own but can take around five years to be cured completely. The long-term outlook for pemphigoid with proper treatment is good. It usually gets resolved after a few years but can relapse at any time, even with appropriate treatment. If any recurring blisters are noticed, one must consult a physician. The majority of the patients respond effectively to the treatment.

8.

Which Doctor Should One Consult for Pemphigus?

Usually, patients consult a general physician. Once the condition is identified as pemphigus, the physician may refer the patient to a dermatologist (a medical expert who specializes in treating the diseases of the hair, skin, and nails). Regular follow-up with the dermatologist is necessary to get relief from the condition.

9.

Is Stress Responsible for Causing Pemphigoid?

Stress is the primary factor that can flare up a pemphigoid. Stress induces antibodies, which in turn can increase the number of blisters. Emotional stress or stressful life events can trigger and escalate pemphigus. Simple exercises, brisk walking, listening to music, deep breathing, meditation, yoga, and swimming can calm the mind, and reduce anxiety and prevent pemphigoid from getting worse.

10.

Can One Lead a Normal Life With Pemphigus?

Pemphigus can resolve on its own after a few years with proper treatment, but there are chances of relapse. The condition can be managed with certain medications. With supportive treatment, the patient can lead a normal life, but if untreated, it can be fatal. Infection of the blisters can result in sepsis (overactive response to infection), which can be life-threatening. With recent medications, the lifespan of these patients is normal. 

11.

Is There an Ayurvedic Treatment for Bullous Pemphigoid?

Though allopathy treatment is the primary mode of treatment for pemphigus, alternative medicine like Ayurveda has been successful in treating bullous pemphigoid. The side effects and high cost of allopathy push the patients to shift to alternative medicine. Studies show that bullous pemphigoid can be successfully treated with herbal medicines.

12.

Can One Die From a Pemphigoid?

Pemphigus, if left untreated, can be fatal. The risk of death from bullous pemphigoid is high in patients with associated medical conditions, advanced age, and in those who are hospitalized. Infected blisters can lead to sepsis (overactive response to infection), which can be life-threatening. In severe cases of bullous pemphigoid, one-year mortality rates can be as high as 25 to 30 percent, even with medical care. 

13.

How Is Pemphigoid Diagnosed?

The gold standard diagnostic test for bullous pemphigoid is direct immunofluorescence (DIF) microscopy of a perilesional biopsy (a type of biopsy in which the outer layer of the skin called epidermis is included). Skin samples from the affected areas may be obtained to detect the presence of antibodies. Blood tests will also be performed to identify the circulating antibodies.

14.

Where Does Pemphigoid Occur?

The blisters can appear on the skin and can involve the mucosal surfaces like the inside of the mouth, nose, throat, eyes, and genitals. Bullous pemphigoid can occur in areas that bend, like the lower abdomen, armpits, and upper thighs. It initially starts as an itchy, elevated rash. As the disease progresses, large blisters are formed on the skin. It can also occur in a few pregnant women.

15.

Is the Brain Affected by Pemphigoid?

Bullous pemphigoid has been found to be linked with cerebrovascular disease (a disease that affects the blood flow in the brain) and dementia (a serious mental issue caused by brain disease or injury that can have an impact on the ability to think, remember, and behave normally). A study shows that psychiatric and neurological (nerve) disorders can have a connection with bullous pemphigoid. 
Dr. Kumari Monalisa
Dr. Kumari Monalisa

Dermatology

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