What is a bullous pemphigoid?
Bullous Pemphigoid Disease: Causes, Treatment, Symptoms, Cancer Pictures: A bullous pemphigoid predominantly affects people over 60 years of age. Men are slightly more affected than women. The bullous pemphigoid is one of the autoimmune diseases: The cause lies in a kind of error of the body’s immune system ( immune system ). It attacks certain components of the skin.
As a result, bulging bubbles form on the skin and, more rarely, on the mucous membrane. In some patients, only swollen redness and lumps are visible. Often sufferers suffer from severe itching. Bullous Pemphigoid Disease: Causes, Treatment, Symptoms, Cancer Pictures
Itching on the skin or itching blisters? Especially in the elderly, these symptoms may indicate a bullous pemphigoid. More about causes, diagnosis and therapy.Important information
What Causes the Bullous Pemphigoid?
When our defense system ( immune system ) comes into contact with constituents of pathogens, it forms certain protein molecules (antibodies). These are detectable in the blood and help to eliminate the foreign substances.
Sometimes, however, the immune system “mistakenly” forms antibodies directed against the body’s own structures. They are called autoantibodies.
In the case of bullous pemphigoid, the body almost accidentally forms antibodies (yellow in the picture) against components of the skin© Thinkstock / iStockphoto
In the case of bullous pemphigoid, autoantibodies are produced against two specific protein molecules in the skin. They are naturally at the interface between the epidermis and the underlying dermis and are responsible for the mechanical cohesion of the two layers of skin.
These two protein molecules are called:
- bullous pemphigoid antigen 1
(BPAg1, also BP230) and
- bullous pemphigoid antigen 2
(BPAg2, also BP180).
If these autoantibodies bind to their target structures in the skin, inflammation occurs. It can be felt in the form of itchy redness and swelling. Inflammatory cells release enzymes at the interface between the epidermis and the dermis, which attach the connection between these layers of skin. This creates gaps just below the epidermis (subepidermal). Tissue fluid enters the fissures and expands them into blistering bubbles.
It used to be thought that cancer could promote the appearance of bullous pemphigoid. Today it is known that patients with a bullous pemphigoid do not suffer from malignant tumors more frequently than other people in the same age group.
What are Bullous Pemphigoid Symptoms
In most patients, sharply defined, partially swollen redness and reddened nodes appear. These itch very much.
Only after weeks or months, in some patients even after years, bubbles are added. These usually develop on reddened skin, but can also occur on normal-looking skin. They are sometimes only a few millimeters small, but can also reach diameters of one to two centimeters or more. Inside the bubbles is clear, slightly yellowish colored tissue fluid. Occasionally some blood is added.
The ceiling of the bladder consists of the entire thickness of the epidermis in the case of the bullous pemphigoid. Therefore, the bubbles are bulging and relatively resistant to mechanical stress. If they are opened anyway, wet, occasionally easily bleeding, superficial skin defects (erosions) develop.
Reddening and swelling, red nodes, blisters and erosions coexist in the full-blown stage of the disease.
Skin Lesion of Bullous Pemphigoid
The skin lesions of bullous pemphigoid may occur all over the body. Especially often they arise in the range of
- Inner thighs
- Flexor sides of the arms.
Only every fifth patient also affects the oral mucosa or conjunctiva. The mucosal changes heal without scars in the case of bullous pemphigoid.
If a bullous pemphigoid is not treated adequately, new skin changes will occur again and again. In some patients, the disease heals after a few years, so that no more therapy is required.
Diagnosis of Bullous Pemphigoid?
The doctor asks the person concerned about his history and examines the entire skin with the naked eye.
He then takes a tissue sample (biopsy) from diseased skin for microscopic examination to rule out other skin diseases . In the case of a fresh bladder, a gap is visible in the tissue. If this is just below the epidermis, at the border to the dermis (subepidermal), the finding fits into a bullous pemphigoid. If, on the other hand, the gap is located within the epidermis (intraepidermal), there is rather no bullous pemphigoid but a pemphigus disease .
For further clarification, the doctor initiates a special examination on a second skin sample: With the aid of the direct immunofluorescence examination (DIF), antibodies can be detected that have bound to structures within the skin. In the case of a bullous pemphigoid, linear deposits of IgG class antibodies are seen along the border between epidermis and dermis.
In addition, the doctor has in the patient’s blood for autoantibodies against the protein molecules BP180 and BP230 search. Control of antibodies to BP180 may also be useful as the amount of these antibodies is in direct proportion to the current disease activity of bullous pemphigoid.
Treatment of bullous pemphigoid?
External Therapy: In mild cases, a purely external treatment with a cortisone preparation (glucocorticoid) can bring the lesions healed. However, it usually comes again and again to new attacks with skin lesions in other places. Therefore, it has been proven to treat already onset redness – according to the doctor – with the glucocorticoid cream. In many patients, the formation of new blisters can be prevented.
Treatment With Drugs : If larger areas of the skin are affected and / or new blisters occur constantly, the doctor additionally prescribes a glucocorticoid in the form of tablets. This acute therapy usually shows a clear effect after only a few days. As soon as the doctor is sure that the existing skin lesions heal and no new blisters appear, he begins to gradually reduce the tablet dose. In many cases, the drug can be discontinued at some point. Occasionally, prolonged therapy with a low, body-friendly glucocorticoid dose is required.Drugs
Further Therapy options
Patients who have multiple bladders at the onset of the disease often also require long-term treatment with what is known as a cortisone-sparing (or even steroid-sparing) drug. This refers to an anti-inflammatory drug that can be given over a longer period of time, but its effect is delayed. As a sole treatment or in combination with a glucocorticoid in the form of tablets, it helps to prevent a renewed surge of disease.
As a steroid-saving drug is, inter alia, doxycycline into consideration. This drug is proven to be an antibiotic, but shows anti-inflammatory effects in a low dosage that is not yet effective against bacteria. Alternatively dapsone may be given, in case of a more severe bullous pemphigoid also azathioprine. During treatment, the doctor will regularly take blood samples to make sure that the patient tolerates the drug well in the long term.
Patients in whom the combination therapy of a glucocorticoid and an immunosuppressive agent is not sufficiently effective, can additionally receive an infusion treatment with special endogenous protein substances (immunoglobulins). This is a mixture of antibodies from the blood of many people, which is given in high doses every four weeks. Those components that have the antibodies in common bind to certain structures (receptors) on the surface of different cells of the immune system. In doing so, they affect the activities of these cells in a complex manner, suppress the inflammatory reaction in the skin and inhibit the formation of autoantibodies.
While this treatment may not be strong enough, the doctor combines therapy with a glucocorticoid and a steroid-sparing substance with a special blood wash. The autoantibodies are removed from the patient’s blood. The involved, very complex procedures are called plasmapheresis or immunoadsorption. Patients with a particularly severe bullous pemphigoid may alternatively receive the substance rituximab. This destroys the antibody-forming immune cells and thus also those cells that are responsible for the autoantibodies against BP180 and BP230.
Some of these medicines are not officially approved for the treatment of bullous pemphigoid. The doctor and the patient must therefore discuss in detail whether the therapy is still suitable in individual cases, which risks exist and which side effects can occur.NOTE
Itchy redness and blisters on the skin or mucous membrane can have many causes. It is not always a bullous pemphigoid. Changes to the skin or mucous membranes should always be clarified by the doctor!Very important :
Classification and disease name
Bullous pemphigoid is the most common disease within the group of autoimmune blistering disorders. This term summarizes different, mostly chronic autoimmune diseases characterized by blistering of the skin and / or mucous membranes.
The term ‘bullous pemphigoid‘ derives from the Latin word bullosus ‘blasig‘ as well as the Greek words pemphix for ‘bubble‘ and eides ‘to be similar’.