Edema of the quinque is inherited
This is an epileptic edema of the skin and mucous membranes in the form of areas of different sizes.
Both sexes are affected approximately equallyoften; the beginning can be at any age, but more often on 3 - 4-th ten years. To make it easier for you to imagine a picture of the disease, I will give a description of one case
The young woman suddenly had swelling of the rear of the hand. He developed very quickly - like those areas of edema that occur after a mosquito bite.
Upon examination, you will see a rounded swellinga few centimeters in diameter. The color of the skin above the edematous place is different in each individual case: it is completely normal, then slightly reddened. then a little yellowish. Consistency is quite dense, and the pits do not remain from pressing. Subjectively, the patient experiences a feeling of tension in this place and sometimes an easy itch. No other symptoms you will not find.
Edema, as I said, developed very quickly - ina few minutes Then for a few hours it grew slightly, a little more time remained unchanged, and then. went on the decline and disappeared without a trace.
In the simplest cases, the picture of an attack can be limited to this. And most seizures can be repeated with different frequency, starting with daily swelling and ending with one seizure in a few years.
This picture represents the simplest type of disease. But in different individuals, individual elements of the clinical picture can vary.
The magnitude of plaque swelling may be different - fromsuch as after a mosquito bite, and up to an edema that occupies a whole section of the limb, for example the forearm and the wrist or even the whole limb. The number of plaques can also be different, ranging from one and up to a decent number in different places. Their localization is the most diverse: limbs, trunk, face. Since there is a lot of loose fiber on the face, swelling here has the peculiarity that a small plaque causes great disfigurement: the eye will swell, the lip and cheek will swell. An important variation in localization is the case where edema occurs on the mucous membranes of internal organs. The plaque can develop in the oral cavity - in the tongue, in the soft palate, palatine arches, in the larynx. In the latter case, a trifling disease turns into a terrible suffering: the laryngeal edema can be so strong that it will require a tracheotomy. And if the surgical help is late, the case may end in death, which is observed from time to time. Edema can occur in the walls of the stomach and intestines: it affects the pain in the abdomen, vomiting, diarrhea. Whether he can be in other bodies, a question disputable. Some go so far as to admit the possibility of such a process in any organ and reduce to it quite a few clinical pictures that are unclear in the sense of pathogenesis. The fact is that in people with edema Quincke there are occasional transient disorders on the part of the kidneys - a protein in the urine, oliguria. Sometimes there are cases when, for no apparent reason, a one-sided pop-eyedness appears for several days, and this is repeated many times. There is an assumption that in such cases, Quincke's edema develops in the orbital fiber and pushes the eye out anteriorly. Sometimes for a short period of time brain phenomena develop - hibernation, headaches, bradycardia, general convulsions; and all this occurs during the rash of plaque edema on the skin. Interpreted: the whole picture in the sense that it is about the focus of the edema somewhere in the cavity of the skull, for example in the shells.
Not so controversial edema in muscles, tendonvaginas and joints. The swelling in the muscles is noticeable to the touch, and they functionally affect the pain, the difficulty of passive and active movements, sometimes temporary contractures. Approximately the same can be said about transient tendovaginitis - edema of tendons and tendon sheaths.
Periodic swelling of the joints - most often knee joints - has even been isolated at one time in an independent picture called hydrops articulorum intermittens.
Now I return to the variations of individualelements of the clinical picture I have already said that the frequency of seizures can in some cases be very different. And the duration of the whole attack also varies very much: sometimes the whole thing ends in a few hours, sometimes it lasts several days.
Everything I have said so far representsthe most common symptoms. Much less frequent seizures are accompanied by a fever. And then there is a series of combinations of a combination of Quincke's disease with migraine, acroparesthesia, erythromelalgia, Reno's disease and finally with urticana. flow. In this sense, we can distinguish two types of disease. In one case, it is a case of occasional rare attacks that are associated with intoxication - among other things, food - and infections. About the flow of such cases much to say is not necessary, they are random, episodic.
The second type presents a picture much morepersistent illness lasts many years, sometimes even tens of years, attacks are frequent, and their causes can not be caught at all. The point here is, therefore, chronic long-term suffering. pathological anatomy knows only a few fragmentary facts. Skin edema due to its simplicity attracted little attention. A little more lucky with the mucous membranes. So. in cases of death from the laryngeal edema, the corresponding places were studied anatomically. An ordinary picture of the edema appeared that did not contain anything specific. With one researcher a rare case occurred, he washed the stomach on the occasion of a picture of the edema in this organ and a scrap of mucous membrane appeared in the wash water. This patch was studied anatomically, and a picture of severe edema was found. Thus, the mechanism of gastric disorders by this case is established quite accurately, and at the same time for other organs the principle possibility of the same process becomes very probable. pathogenesis. Despite the simplicity of the clinical picture, its mechanism does not lend itself to explanation at present, since here we are faced with one unsolved fundamental issue. This is the question of the ultimate cause of edema in general. There are three possibilities:
1) Vascular dysfunction. The walls of the vessels for some reason lose their normal impermeability and are made passable for blood plasma.
2) Excessive formation of lymph, which does not fit in its natural channel and permeates surrounding tissues.
3) Tropism of tissues in relation to water, - something like hygroscopicity.
Which of these three possibilities doesQuincke's disease. completely unknown. But what is especially important, it is not known what dependence on the nervous system these factors are. Meanwhile, the last point is the center of gravity of the question: Quincke's disease is considered a neuropathic edema. Is this true? Is it not usual for physicians to dump everything they do not understand on the nervous system?
Apparently, after all, the connection with the nervous systemthere is. Some facts are said for this, although we must confess. that there are not many of them. The most spectacular of them is the observation of one neuropath, suffering so-called agoraphobia - obsessive fear to cross the square. When he had to make such a transition alone, he developed a swelling of Quincke; when he went with a doctor, there was no swelling.
Sometimes, as evidence of the nervous nature of edema, there are cases where the disease developed after a trauma. This is not so convincing - it may be a coincidence.
But especially convincing argument for participationThe nervous system is the fact of heredity of the Quincke edema. Indeed, this disease sometimes affects several members of the same genus and flares up in several generations. There were, for example, cases when there were data on 5 generations of the same genus, where there were only 15 healthy and 21 with Quincke's edema. Or such a genealogy: traced 3 generations, where there were 47 healthy and 33 with edema Quincke; of the last 12 died of laryngeal edema.
Incidentally, in such surnames, apparently, there isa tendency to swelling of the larynx, which should be taken into account when assigning, for example, an operation in the mouth: there is a case when such a patient was removed tonsils and he died from an attack of the laryngeal edema.
Such families, as I said, are considered especiallyEvidence in the sense of the neuropathic nature of Quincke's edemas: they are inherited as if those properties of the nervous system that favor edema. This is a big misunderstanding. With the same right, one can think of a hereditary transfer of the features of the circulatory system, the lymphatic, hygroscopic, so to speak, properties of tissues, etc.
I sum up all this: apparently the nervous system can play a role in the mechanism of the edema of Quincke.
I said that the clinic suggests divisiondiseases of two types. It is believed that the pathogenesis of these two types is different, in particular, in the second type, the primary role is given to the nervous system. You have seen that all the arguments here operate almost entirely with many unknowns. Therefore, an attempt to distinguish two pathogenesis of the disease must be considered premature.
End the question of the mechanism of suffering to mewill be the same as where I started: it is at present precisely unknown. treatment and prognosis. It is important to appoint the right diet for the patient: it is noticed that the milk-vegetarian table and the systematic consumption of sour milk (yogurt or lactobacillin) soften the manifestation of the disease. Not bad results give arsenic and strychnine. In some cases, atropine helps. prediction for cases of the so-called second type is serious: the disease can last for years. In family cases, there is a danger of death from the laryngeal edema.