According to electronicsencyclopedia.com, Linear IgA dermatosis is an autoimmune disease of the skin in which the body’s own immune system produces autoantibodies against adhesion proteins. The result is blistering and reddening of the skin, which in individual cases can also affect the eyes. If the eyes are involved, there is a risk of blindness, which can also necessitate invasive treatment methods in addition to drug therapies.
What is linear IgA dermatosis?
The immune system serves to recognize and defend against foreign substances and pathogens. In autoimmune diseases, on the other hand, the immune system directs antibodies against the body’s own tissue. In principle, autoimmune diseases can attack any type of tissue.
If the skin tissue is the target of the autoimmune antibodies, we are talking about autoimmune dermatoses. One such dermatosis of autoimmune origin is linear IgA dermatosis, also referred to in the medical literature as IgA pemphigoid or chronic bullous dermatosis of childhood. The disease is accompanied by erythema and blistering.
It is an extremely rare disease that primarily affects women and young children. Among the autoimmune dermatoses, the disease can be assigned to the subcategory of blistering autoimmune dermatoses because of the associated blistering. So far, one can only speculate about the primary cause of linear IgA dermatosis. The annual incidence of the disease in Germany is less than one case per 100,000 inhabitants.
Although the primary causative factors for autoimmune diseases are still the subject of speculation, at least the pathophysiology can be explained to a large extent. The linear autoimmune IgA dermatosis represents an incorrect programming of the immune system, which goes back to an unexplained genesis. Based on the incorrect programming, the body’s own defense system forms autoantibodies in the serum.
These autoantibodies are directed against a protein in the intracellular plaques of hemidesmosomes. Hemidesmosomes are adhesive complexes that firmly attach epithelial cells to the underlying basement membrane. With the destruction of important proteins in the intracellular plaques of the adhesive complexes, adhesion is lost.
At the same time, antigen-antibody complexes form along the basement membrane, which activate the complement cascade. This activation produces the characteristic erythema of linear IgA dermatosis. Scientists now suggest that the wrong programming of the immune system in this case could be induced by medication, for example.
Symptoms, Ailments & Signs
Patients with linear IgA dermatosis characteristically suffer from multiple blisters of the outer skin. In most cases, the blisters do not appear on healthy skin, but form on very reddened areas of skin in the sense of erythema. In some cases, erythema and blisters coexist.
In still other cases, blisters detach the erythema and vice versa. The occurrence of blistering on the mucous membranes is not completely excluded, but rather not characteristic. The skin changes in patients are usually accompanied by more or less severe itching, burning sensations or pain.
Many patients scratch the blisters to relieve itching. However, this approach can make the lesions worse. The skin changes occur most frequently on the proximal extremities or in the trunk area. Participation of the eyes has been documented in isolated cases. This ocular involvement can lead to blindness due to the resulting scar tissue or result in the formation of entropion.
Diagnosis & disease progression
The diagnosis of linear IgA dermatosis is typically based on immunohistopathology. The autoantibodies must be isolated from the skin in order to make a diagnosis in order to confirm an existing suspicion of autoimmune dermatosis. The first suspicion comes to the dermatologists usually through visual diagnostics.
To confirm the diagnosis, he secures the linear IgA and complement deposits on the basement membrane. In some cases, the specific autoantibodies can even be detected in the patient’s serum. Therefore, laboratory diagnostic evidence can also be considered. However, the disease is by no means excluded if the specific antibodies cannot be detected in the serum.
In terms of differential diagnosis, linear IgA dermatosis must be differentiated from skin diseases such as dermatitis herpetiformis Duhring or bullous pemphigoid. The prognosis cannot be generalized for patients with IgA dermatosis. Typically, autoimmune diseases are characterized by an individual course.
In this disease, those affected primarily suffer from blisters on the skin. These can lead to reduced aesthetics and thus significantly reduce the patient’s quality of life. Inferiority complexes and low self-esteem appear. It is not uncommon for those affected to develop depression or other mental disorders.
The disease also causes itching or burning pain on the skin. If this pain also occurs at night, the patient may have difficulty sleeping and become irritable. Complications usually arise when the disease also affects the eyes. In the worst case, the affected person can go blind. The permanent pain and discomfort on the skin severely restricts the patient’s life.
In most cases, the treatment of the disease is carried out with the help of medication and leads to success. Not infrequently, however, patients are dependent on lengthy treatment. Even after treatment, this disease can develop again. The life expectancy of the patient is usually not affected.
When should you go to the doctor?
Anyone who notices blisters, redness and other skin changes should seek medical advice promptly. Linear IgA dermatosis, although very rare, can have serious health implications if treated late or not treated at all. Affected people should therefore have even inconspicuous skin changes examined by a doctor. If there is pain, itching or crusting, it is best to see a doctor on the same day.
Mucosal adhesions in the eye area must be treated immediately. Otherwise, you may experience severe pain and blurred vision. Linear IgA dermatosis occurs primarily in children. Risk factors are infections or tumor diseases. The disease often occurs in connection with a chronic inflammatory bowel disease such as Crohn’s disease.
Affected people should consult their family doctor or a dermatologist. Any visual disturbances must be presented to the ophthalmologist. If mental problems develop as a result of the skin changes, you should speak to a person you trust. If in doubt, we recommend talking to a psychologist.
Treatment & Therapy
Patients with IgA dermatosis receive a combination of external and internal therapy. External therapy includes anti-inflammatory treatment, which can be done with synthetic tanning agents, for example. Internal therapy is carried out with agents such as DADPS.
Depending on the current situation and the course of the individual case, the medication and its dosage are adjusted piece by piece. If the situation remains unchanged, azathioprine and systemic corticosteroids can be considered. In addition, cyclophosphamide, colchicine, mycophenolate mofetil, methotrexate, and ciclosporin A are internal treatment options.
In principle, linear IgA dermatosis responds well to immunosuppressants as well as sulfones and sulfapyridines. Immunosuppression dampens the entire immune system and thus also reduces the formation of autoantibodies. All immunosuppression measures can be used in the form of long-term therapy. Other active ingredients are usually more suitable for acute therapy.
Systemic treatment of the itching can be achieved by the systemic administration of various antihistamines. In contrast to many other autoimmune dermatoses, dietary measures are largely ineffective in autoimmune IgA linear dermatosis. In case of ocular involvement, invasive measures may be necessary. Especially after the formation of an entropion, an ophthalmological correction is necessary.
Outlook & Forecast
When seeking medical care, the prognosis of linear IgA dermatosis is favorable. The defect in the immune system can be well regulated by administering medicines with the current medical possibilities. If left untreated, the symptoms persist or increase in intensity or extent. In addition, mental disorders are to be expected, so that many of those affected experience severe emotional stress due to the visual abnormalities. The earlier a diagnosis is made, the sooner treatment measures are possible and relief recognizable.
Despite the good therapeutic options, side effects can occur due to the prescribed preparations. These have a delayed effect on the healing process, since a restructuring of the treatment plan is necessary. Although the disease can be treated well with existing drugs, long-term treatment is necessary in most cases.
The disease recurs in most patients as soon as the drugs are stopped. For this reason, those affected have to undergo medical treatment for several months or years. This circumstance can also have a negative effect on the psyche of the person concerned. Depending on the personality of the patient, possible psychological sequelae develop. If the disease progresses unfavorably, the affected person becomes blind. In these patients, the prognosis is significantly worse.
The primary triggers of incorrect programming of the body’s own immune system remain the subject of discussion to this day. If, as speculation has it, different drugs actually trigger IgA dermatosis, these drugs could be avoided as a preventative step. On the other hand, promising alternatives are not available for all medications.
The aftercare measures for skin diseases usually depend very much on the exact disease, so that no general prediction can usually be made. However, an IgA dermatosis must first and foremost be examined and treated by a doctor so that there are no further complications or symptoms. The earlier this disease is recognized and treated by a doctor, the better the further course.
For this reason, it is advisable for those affected to consult a doctor as soon as the first symptoms and signs appear. A high standard of hygiene also has a positive effect on the course of such diseases in most cases. In most cases, these diseases are treated by applying creams or ointments and taking medication.
Those affected should pay attention to regular use and the right dosage in order to permanently relieve symptoms. Regular check-ups by a doctor are very important. In most cases, the skin diseases do not have a negative effect on the life expectancy of the affected person. Contact with other patients can also be useful.
You can do that yourself
It is not possible to treat linear IgA dermatosis with self-help methods. Those affected are therefore always dependent on medical treatment to prevent further complications and symptoms. First and foremost, early diagnosis and treatment are therefore necessary, as these always increase the chance of a positive course of the disease. If the first symptoms of linear IgA dermatosis appear, a doctor should be consulted immediately.
Since linear IgA dermatosis is mainly treated with medication, those affected should always ensure the correct dosage and regular intake. Interactions with other medications should also not be ignored.
If blindness has occurred, the patient is dependent on help in his everyday life. This help should ideally be provided by relatives or friends and relieve the patient in his everyday life. Empathetic conversations with close friends or other people you trust can often help to avoid and alleviate psychological problems. The same also applies to psychological complaints that occur due to the reduced aesthetics caused by the skin complaints.