As bullous dermatoses are a group of skin diseases in which the body makes antibodies against the structures in the skin so that the skin dissolves and bubbles form. These antibodies are directed against components of the upper skin ( epidermis), the so-called desmosomes, or to components of the basement membrane separates the epidermis and dermis of each other.
- 2.1 therapy
We distinguish the following subgroups:
The antibodies directed against the epidermal desmosomes, special protein molecules that connect the individual keratinocytes ( corneocytes ) together. A distinction is still pemphigus vulgaris, are involved in the rather deep layers of the epidermis, so that larger flaccid bullae arise that contain some liquid, pemphigus foliaceus, are involved in the superficial layers of the epidermis, so that the skin puff like peeling and no real bubbles arise, as well as other, very rare Pemphigusformen. Pemphigus are very rare in Central Europe and mainly patients about the sixth decade of life.
Here it comes to loss of contact in the area of the basement membrane. The antibodies directed against the hemidesmosomes, protein molecules that connect the bottom keratinocyte layer with the basement membrane. Again, bubbles form because the bladder blanket but is formed by the entire epidermis, the bubbles are filled taut and bulging in pemphigoid diseases. The most important is the bullous pemphigoid Pemphigoiderkrankung, which is much more common, often than suspected, as show even before the bubble formation many skin symptoms that are often treated as eczema or psoriasis. There are also other forms of pemphigoid, however, are rare. In this group there are also linear IgA dermatosis and epidermolysis bullosa acquisita. Allen diseases mentioned so far - other than the M. herpetiformis Duhring common that primarily IgG antibodies are formed.
Duhring 's disease / dermatitis herpetiformis
The Duhring 's disease occupies a special position, as the blistering takes place here a little deeper. The occurring in this disease IgA type antibodies are directed against the epidermal transglutaminase. These occur especially on the extensor surfaces ( elbows ) solid bubbles urticae and papules (skin nodules ) on. Typical is the unbearable itching. Be provoked relapses of the disease by gluten ( gliadins ) and iodine ( sea fish ).
Duhring 's disease is the cutaneous manifestation of celiac disease ( sprue, gluten intolerance ). That is, not all celiac disease occurs with Crohn Duhring, but everyone with Crohn's Duhring has a sprue. However, this must not be clinically imperative to recognize. A lifelong gluten- free diet is still a cornerstone of therapy of this disease dar.
Epidermolysis bullosa acquisita
Here, antibodies are directed against the collagen 7, a long-chain protein that connects the basement membrane to the underlying connective tissue of the dermis. The disease is very rare. The bubbles are tight as in bullous pemphigoid, but it rarely comes to the formation of bubbles, but often other symptoms such as injured skin and disorders of the nails.
In the blistering autoimmune one prove the binding of the antibodies in the skin. Here a small sample not infected skin is recovered and treated with a special fluorescent dye that binds to the antibody. Then it looks at the incision with a fluorescence microscope to view the distribution pattern of the antibody. Thus we find, for example, pemphigus in a net-like deposit in the epidermis, while pemphigoid the antibodies are provided in a line at the basement membrane. This detection of antibodies directly in the tissue are referred to as direct immunofluorescence. If you want the antibodies detected in the blood, you can mark it in the serum and then apply on specially prepared tissue sections of animal and human origin. Here, the evidence of bullous autoimmune dermatosis is not always possible. The advantage is one that a serum sample is much easier to win than a skin sample can be send also lighter and also on specially prepared skin is also a distinction between pemphigoid and epidermolysis bullosa is possible. This is also known by the indirect immunofluorescence. Disease activity also can not track more accurately in some cases by means of an ELISA.
As with many autoimmune diseases, here too the therapy with drugs that must weaken the immune system. Here, in addition to agents such as cortisone and azathioprine, mycophenolate mofetil or dapsone can be used. In severe cases, the antibodies can also be removed by means of a type of hemodialysis ( immunoadsorption ) from the blood. This may also be necessary to give other medications to prevent the formation of antibodies.