Cicatricial mucosal pemphigoid is a chronic bullous dermatosis. It shows up primarily on the mucous membranes.
What is cicatricial mucosal pemphigoid?
Scarring mucosal pemphigoid is an autoimmune disease characterized by the formation of skin blisters that become chronic. The word pemphigoid is borrowed from the Greek term pemphix and means “bubble”. In addition, the mucous membrane is involved in the clinical picture. For all you need to know about capsule rupture, please visit phonecations.com.
The blisters often show up on the body together with superficial skin wounds. Scars will appear later as the wound heals. In earlier years, the use of the term “scarring mucosal pemphigoid” was common in medicine.
Today, however, doctors only use it for a rare form of disease in which there is no predominant involvement of the mucous membrane and in which the body heals with scarring. Instead, the term mucosal pemphigoid is used.
Furthermore, the disease represents a special form of bullous pemphigoid, which also belongs to the blistering skin diseases. The scarring mucosal pemphigoid is one of the rare diseases. Around two people out of a million people get sick every year. Elderly people in particular are affected by mucosal pemphigoid.
Causes
Scarring mucosal pemphigoid is an autoimmune disease. What causes it is still unclear. There was no evidence of eating or taking medication as a trigger.
Responsible for the symptoms of the scarring mucous membrane pemphigoid is the organism’s immune system, which is directed against the body’s own components of the skin. Special proteins are formed that are known as autoantibodies. The antibodies attack some proteins located in the basement membrane.
The membrane forms the connecting layer between the epidermis (upper skin) and the dermis (corium). The proteins attacked are laminin 332, α6ß4 integrin, BP 180 and BP 230. Approximately 25 percent of all patients in whom laminin 332 is attacked develop cancer as the disease progresses. For this reason, the diagnosis plays an important role in the early detection of the tumor disease.
Symptoms, Ailments & Signs
The oral mucosa and the mucous membrane of the eyes are particularly affected by a scarring mucosal pemphigoid. Sometimes the disease can also appear on the pharyngeal mucosa, nasal mucosa, genital area or anal region, but this happens less frequently. The skin is only involved in about 30 percent of all patients.
Typical features of scarring mucosal pemphigoid include painful changes in the oral mucosa. These often result in problems eating, which in turn manifests itself in weight loss and feelings of weakness. However, the extent of the disease varies from person to person.
While cicatricial mucosal pemphigoid begins in the oral mucosa in most people, it may first appear in the nasal mucosa in some patients. This leads to nosebleeds, problems breathing through the nose and the formation of bloody crusts. In women, there are often complaints of the genital mucosa.
Because the blisters that form on the mucous membranes tear easily, this often leads to painful sores. A scarring mucous membrane pemphigoid in the eyes is considered to be particularly alarming, since in extreme cases blindness due to secondary corneal changes is possible.
When the blisters burst, scarring shrinks, which at the same time leads to chronic conjunctivitis ( conjunctivitis ). In addition, there are limitations in eye movements. There is also a risk of obstruction of the tear ducts caused by the formation of scars. This can dry out the conjunctiva, leading to the risk of losing the eye.
Diagnosis & course of disease
The doctor receives the first indications of a scarring mucosal pemphigoid when he makes his diagnosis from the characteristic symptoms of the disease. A blood test and the removal of tissue are required for a more precise diagnosis. There is the option of using a special staining technique to detect the responsible autoantibodies in a tissue sample taken from the mucous membrane of the cheek.
Deposits of the antibodies can be seen at the border area between the mucous membrane and the connecting layers located below it. It is also possible to detect antibodies in the blood. For this purpose, special tests such as ELISA, indirect immunofluorescence on split skin tissue or Western blot are carried out.
The scarring mucosal pemphigoid takes its course in spurts, which can last for months or even years. Problems arise primarily from the scarred blister healing and visual disturbances when the eyes are involved.
Complications
Those affected suffer from a variety of symptoms with this disease. The exact symptoms depend very much on the affected region, so that no general prediction is possible. In most cases, there is severe pain due to the change in the mucous membranes. Those affected sometimes suffer from weight loss and permanent tiredness and exhaustion.
You feel weak and can no longer carry out usual activities. It is also no longer easy to take in food and drink, so that many of those affected suffer from deficiency symptoms or dehydration. The quality of life is significantly reduced and limited by the disease.
There are also nosebleeds and frequent breathing difficulties. In the worst case, the person affected can lose consciousness completely. If the disease spreads to the eyes, it can also lead to blindness.
This disease is treated with medication or dialysis. Although there are no particular complications, complete healing is usually not possible. Life expectancy is also significantly reduced by the disease in most cases.
When should you go to the doctor?
Mucosal abnormalities should be reported to a doctor if they persist or worsen over several days or weeks. Irregularities in the eyes, breathing, swallowing cycle and excretions are signs of a health problem. A doctor’s visit is necessary to clarify the cause. Discomfort in the mouth or a general feeling of illness indicate a disease. Loss of weight, an inner weakness or a loss of physical performance are considered to be worrisome. Consultation of a doctor is necessary if functional disorders occur or involuntary bleeding occurs. In the case of repeated, uncontrolled and incomprehensible nosebleeds, research into the cause is advisable.
This process is considered a warning signal from the organism. Painful vision or restricted vision can indicate problems in the mucous membrane of the eye. To avoid complications, a doctor’s visit should be made early. Anxiety, sleep disorders and respiratory restrictions should also be discussed with the doctor treating you. Problems with the tear ducts, dryness in the eyes or abnormalities in general eye movements are signs of a disease. If wounds develop on the body in the area of the mucous membranes or if pain occurs, treatment should be carried out. If there is a refusal of food supply because of the impairments, a doctor is needed.
Treatment & Therapy
The therapy of the scarring mucosal pemphigoid depends on whether the conjunctiva of the eye is also affected by the disease or not. In most cases, a hospital stay is required at the beginning of treatment. It is important to fight the inflammation of the mucous membrane and to suppress the autoantibodies.
In the acute phase, the patient receives medication such as cortisone preparations as well as azathioprine, dapsone, cyclophosphamide, sodium mycophenolate or mycophenolate mofetil, whereby these active ingredients can sometimes be combined with one another. If, in severe cases, the drugs administered do not have the desired effect, an immune adsorption can take place, which is a special form of blood washing.
Rapid therapy is necessary, especially in the case of eye involvement, in order to counteract permanent visual damage. To suppress the immune system, the patient receives cyclophosphamide in addition to cortisone.
Prevention
Prevention against scarring mucosal pemphigoid is not possible. So the causes of the autoimmune disease could not be found out.
Aftercare
The scarring mucosal pemphigoid requires intensive follow-up care after therapy. This serves to protect the eyes and avoid permanent visual damage. Therefore, those affected must monitor their symptoms closely so that they can seek medical help immediately if problems arise. The use of special eye ointments and drops must be carried out strictly according to medical recommendations.
Patients often suffer from scarring of the bladder and impaired vision. This can frighten them, but with the right visual aids, the biggest problems can be alleviated. Loving support in everyday life also helps through the most difficult times. Those affected should take it easy during the sick leave, which lasts several weeks, so that the illness can subside completely.
If inflammation or bleeding occurs in the eyes or in the mouth area, it is advisable to see a doctor immediately. This also applies to very painful reactions or functional failures. For aftercare, some doctors recommend resorting to alternative means. A self-help group in which naturopathic and homeopathic therapies are an important topic is also helpful. The exchange with other patients is about mutual support and tips on the one hand and moral cohesion on the other.
You can do that yourself
Scarring mucosal pemphigoid can be treated by those affected themselves. The most important self-help measure is the correct application of the prescribed ointments and brushes. Prescribed retinoids or immunosuppressants must also be taken according to the doctor’s instructions in order to rule out complications.
The impairment of vision must be compensated with the help of a visual aid. Affected people need help in everyday life and have to take sick leave for a few weeks until the condition has completely subsided. If bleeding or inflammation of the eyes or mouth occurs, the doctor must be informed. This is necessary in particular in the case of severe pain or functional failures. Signs of blood poisoning must be clarified immediately. It is best to call the emergency doctor immediately so as not to lose any more time.
A scarring mucosal pemphigoid can also be treated with homeopathic and naturopathic remedies. Homeopathy offers Alumina, Euphorbium, Galphimia and Ginseng. Various medicinal herbs, oils and Schuessler salts are recommended from naturopathy. The use of alternative means should be discussed with the responsible dermatologist or internist. Finally, patients can turn to a pemphigus and pemphigoid self-help group, which is available in all major cities in Germany.
