Lyell's disease
Contents:
Description:
Other names of pathology - an acute epidermal necrolysis or a toxic epidermal necrolysis. This serious illness which has a toksiko-allergic origin i.e. pathological changes in an organism of the patient are caused not only directly allergic reaction, but also defeat of an organism by the toxic substances which are formed during an allergic inflammation. It is characterized by skin blanket amotio from underlying, further there is its necrosis.
Lyell's disease symptoms:
The disease most often affects children and young people. All signs develop during very short period. Already 1-3 days later from the beginning of a disease the condition of the patient becomes extremely heavy, in these terms there can occur even death. Suddenly, without the reason seen on that, body temperature increases to 39-40 °C, sometimes above. Developing then rashes cover skin of a trunk, hands and legs. They are very plentiful, multiple and represent reddening spots with sites of hypostasis of the small sizes.
Gradually they increase, as if spreading on skin, merge among themselves. As a result on a body the large centers of defeat are formed. About 2 days later on the affected skin in the field of the centers the bubbles having various sizes begin to appear. Size them in some cases can reach the patient's palm. The skin covering them flabby, is thinned, is very easily torn at a mechanical injury. Later quite short time all skin of the patient takes such form as though it was scalded by boiled water. The same picture is characteristic of a burn of the II degree. All skin accepts red color, at touch to it sharp morbidity is defined. During a touch it is very easy to shift it, it shrivels, i.e. the so-called symptom of "the moistened linen" comes to light. On fingers a foot and brushes the skin blanket very easily exfoliates and disappears, completely keeping their form. Emergence of a large number of very small hemorrhages on skin of all body is in certain cases possible.
A little the picture which develops on mucous membranes of an oral cavity differs from above described. Here in a large number there are superficial defects which are painful at touch, very easily bleed when traumatizing. On lips there are just the same educations, becoming covered further by rough thick crusts that considerably complicates meal of the patient. Pathological process at a Lyell's disease affects not only an oral cavity, but also mucous membranes of other areas: the throat, a throat, a trachea, bronchial tubes, a gullet, a stomach, intestines, a bladder and an urethra can be surprised. Very often also pathological centers appear on conjunctivas and in external genitals that is shown in the form of inflammatory processes in these areas.
When examining under a microscope of the material received from the patient it turns out that all blanket of his skin is exposed to death, and directly in it there is a large number of large bubbles more deeply. At a research of their contents it turns out that it is absolutely sterile, i.e. does not contain pathological microorganisms at all. Deeper layers of skin edematous, in them a large number of the immune cells migrating here from a blood-groove is located. Their biggest accumulations are around skin vessels. The wall of the last also strongly bulks up.
Overall health of the patient in the course of a disease worsens very quickly, literally in the eyes. Eventually, it becomes extremely heavy. To very big indicators body temperature increases, there are severe headaches, orientation in space and time is broken. The patient becomes sleepy, symptoms of dehydration of an organism appear: constant painful thirst, reduction of amount of saliva and sweat, a pachemia which leads to disturbance of a normal blood-groove in bodies and fabrics, cardiac performance and kidneys. Toxic defeat of an organism of the patient is also shown in the form of a hair loss and nails. In some cases at the patient simultaneous very strong damage of such vitals as a liver, lungs, kidneys is noted. Weight of a condition of the patient with a Lyell's disease is explained by such factors as organism dehydration, disturbance of balance of water, salts and mineral substances, toxic defeat decomposition products of fabrics of own bodies, disturbance of transformation in an organism of proteins, uncontrollable processes of their disintegration. In bodies and body tissues a large amount of proteins of the small sizes which are formed in the course of disintegration of larger proteic matters collects.
The quantity of leukocytes in blood increases in very considerable degree that speaks about strongly expressed inflammatory processes.
Concerning diagnosis, control of a condition of the patient and quality of the carried-out therapy various laboratory researches are very informative. At their carrying out it is possible to reveal increase in blood of quantity of leukocytes, emergence of their unripe young forms, the increase in a blood sedimentation rate which is also a sign of inflammatory process, increase in coagulability of blood, decrease in it in protein content and nutrients, but at the same time increase in proteinaceous antibodies, increase in amount of bilirubin (abnormal liver function), urea (renal failure), nitrogen, increase in activity of some enzymes in blood.
At a disease function of kidneys is strongly broken that can be revealed at a research of the analysis of urine. In urine the amount of protein increases and the blood impurity speaking about increase in permeability of the renal filter come to light.
If the patient does not receive due medical care in time, then his state continues to worsen, the disease develops further, its signs more and more accrue. At the same time development of a number of heavy complications, such as a fluid lungs, death of tissue of kidneys, an inflammation of a renal pelvis, pneumonia, accession of infectious complications is possible. The last is the most adverse as immune forces of an organism of the patient are very strongly weakened, as a result pathological microorganisms are capable to get into a blood stream, to breed there, extending in all bodies and systems. At such picture death of the patient is very probable. Bacteria in blood in a large number allocate toxic products of the metabolism that can lead to development of a depressed case. The most critical period during which the above described complications develop is the 2-3rd week from the moment of development of a disease. During this period of the patient needs especially attentive observation and intensive therapeutic actions. Now, when techniques of treatment of a Lyell's disease are most perfect, are carried out using the most modern medicines, mortality of such patients remains quite high and makes about 1/3 part of all diseased. The probability of death very strongly increases when the diagnosis of a disease was made too late, when performing illiterate treatment, at death more than a half from all integument of the patient. In diagnosis of an epidermal necrolysis the main role belongs to inquiry, survey of the patient, assessment of these laboratory researches.
Very often the dermatologist is faced by a question of what medicinal substance led to development of a disease. At assistance it is necessary to use these or those medicamentous drugs, however, using the drug which caused an allergy in this patient it is possible to lead to considerable deterioration in its state. With the diagnostic purpose at these patients statement of immunoassays is applied. At the same time in the presence of provocative medicinal substance intensive reproduction of immune blood cells begins.
Sometimes the Lyell's disease should be distinguished from such similar diseases as a violent kind of a mnogomorfny exudative erythema, a pemphigus, etc.
Lyell's disease reasons:
The main and most important role in development of pathology belongs to various medicines. On the frequency of the caused allergic reactions sulfanamide antibacterial agents are on the first place. Then on decrease follow: penicillin, tetracyclines, eritromitsina, anticonvulsant drugs, the antiinflammatory, anesthetizing, antituberculous remedies. In the present time cases of development of heavy reaction to use of such "harmless" drugs as vitamins and dietary supplements, means for X-ray contrasting, antitetanic serum, etc. are recorded and described.
Treatment of the Lyell's disease:
First, at emergence of the first symptoms of the considered pathology are necessary the introduction termination for the patient of allergogenny medicine and its immediate delivery in a hospital, it is desirable in intensive care unit at once. Here further active fight against defeat of an organism is made by toxic decomposition products of fabrics and proteic matters, disturbance of coagulability of blood, support of balance of water, salts and other mineral substances, proteinaceous balance, and also a normal functional condition of vitals is made. Let's describe below some techniques of an intensive care which are applied in the following cases and use various ways of purification of blood of toxic substances.
* Extracorporal hemosorption. Is one of the most effective remedies applied for the purpose of therapy of a Lyell's disease. This technique is early and has to be carried out whenever possible in the first 2 days from the beginning of a disease as in these terms is the most efficient. If treatment is begun in time, then after 2 - 3 specified procedures there is almost absolute recovery of the patient. After 3-5 days from an onset of the illness the condition of the patient becomes much heavier, signs of toxic defeat of an organism manage to accrue. In these cases holding 5-6 sessions of the procedure between which it is necessary to do small breaks is already necessary.
* Plasma exchange. This procedure affects the patient's organism in two directions at once. First, it promotes removal from an organism of toxic products which own and bacterial toxins, allergens circulating in blood cell-bound immune complexes, active immune cells are. Secondly, during the procedure normalization of the broken host defense of an organism is made. In early terms the procedure is also carried out 2-3 times, at the same time also other medicamentous techniques of treatment are used. After that the condition of the patient is normalized.
* The therapy directed to normalization and maintenance of balance of water, salts and proteic matters. This direction of treatment of such patients is very important as the specified pathological shifts in many respects define weight of a condition of the patient and the further forecast. Liquid, as a rule, in the volume of 2 l a day constantly has to be entered into an organism. Various special solutions are for this purpose applied to intravenous injections. This action is quite simple, however it is capable to improve considerably the forecast concerning recovery and further life of the patient. It is necessary to remember that at introduction to an organism of large amounts of liquid it is always necessary to watch a regularity and volumes of an urination only.
* Fight against disintegration of skin and own body tissues. With these purposes the medicines containing the substances suppressing work of the enzymes responsible for destruction of fabrics are administered.
* Drugs of adrenal hormones. Their use is obligatory at patients with a Lyell's disease. These substances possess the strongest antiallergic and antiinflammatory action. For the period of the acute period of a disease the highest doses of these drugs which then gradually decrease are appointed. In the first days only injection forms of hormones have to be applied. Further, after stabilization of a state, they are appointed in tablets.
* The drugs supporting functions of a liver and kidneys.
* Antibiotics. Are appointed with the preventive purposes and at accession of infectious complications. It is necessary to avoid very strictly purpose of drugs if in the past the patient on them had allergic reactions.
* Mineral substances. Appointment them quite reasonable and directly follows from the mechanism of development of the disease. Drugs of calcium, potassium, magnesium are appointed generally. Diuretic drugs are at the same time appointed.
* The substances reducing coagulability of blood.
* Hyperbaric oxygenation. The modern technique is that the patient is located in the special device in which there is an oxygen under supertension. Use of this technique for patients with a Lyell's disease very well proved.
All patients need constant observation of work of vitals, especially cardiovascular system. It is made in intensive care unit by means of the special equipment.
The leading role belongs to care of such patients. In chamber where they are, it shall be warm, it has to be by all means equipped with germicidal ultra-violet lamps. It is desirable to place here also special warming framework which is applied at patients with burns. To the patient plentiful drink, liquid food constantly move. Bandages and linen of the patient have to be absolutely sterile.
Directly on area of defeat on skin various aerosols with the adrenal hormones, drugs promoting healing of wounds, antibacterial drugs are applied. For a mucous membrane of an oral cavity rinsings by broths of herbs to which carry a camomile, a sage, etc. are appointed. Greasing of defects by an egg yolk is made for acceleration of processes of healing.
