Polymorphic thermal angiitis
Contents:
Description:
This kind of a skin vasculitis proceeds a long time, at the same time continuous alternation of the periods of aggravations and improvement of a condition of the patient is noted. Even after, apparently, full permanent treatment symptoms of pathology can appear again. Distinctive feature of a disease is that skin manifestations at it are extremely various. Except skin there can be also other most various manifestations.
Symptoms of a polymorphic thermal angiitis:
At a polymorphic (diverse) thermal angiitis, as well as at other kinds of vasculites, the defeat centers first of all develop in areas of shins, however at atypical options of a current they can appear also in other places. Seldom, but nevertheless they can initially be located on mucous membranes of an oral cavity. As it was already specified, rashes at the same time can be the most various, however such educations as the blisters, spots of hemorrhages, small knots and plaques of inflammatory character, big nodes located superficially directly under skin, educations as a cutaneous dropsy with sites of its necrosis, small bubbles, large bubbles, pustules, superficial defects of skin, necrosis of its upper layers, sores and ulcers, cicatricial changes most often meet.
In certain cases emergence of the above-stated rashes is accompanied by such general manifestations as fervescence (sometimes to considerable indicators), weakness, fatigue, weakness, a pain syndrome in joints, headaches. After emergence on a body of rashes they, as a rule, exist further on a body during very long time, from several weeks to several months and even more. After healing of all centers it would also seem an absolute recovery always there is a risk of development of a disease again.
As rashes at the specified pathology can as it was already stated above, have the most various character, depending on dominance of this or that type of elements it is accepted to allocate several independent kinds of a polymorphic thermal angiitis:
urtikarny type at which blisters prevail;
the hemorrhagic type - a major factor in pathological process is developing of hemorrhages;
papulonodulyarny type - sites of puffiness of skin and small knots prevail;
papulonecrotic type at which sites of hypostasis with sites of necrosis of skin are generally observed;
pustular and ulcer type at which the leading elements are ulcers and pustules;
necrotic - the ulcer type proceeding most hard and which is characterized generally by emergence of ulcers (sometimes very deep and very big sizes) in combination with sites of necrosis of skin;
polymorphic type at which take place all or almost all above-stated types of rashes.
Urtikarny type. At most of patients this type to posvoy external manifestations bears to a chronic small tortoiseshell a strong resemblance that in some cases can even serve as the reason of statement of the wrong diagnosis. On a body of the patient there are blisters of the most various sizes which can be located on any body parts. They differ from those at a small tortoiseshell in the fact that are very resistant and never pass quickly. As a rule, they remain on skin of not less than 1-3 days, and often and much longer. At a small tortoiseshell the main complaint of the patient is rather intensive itch in the field of pathological educations while at urtikarny type of the described disease the burning or a peculiar unpleasant feeling connected with irritation of integuments generally is noted. The general disturbances are noted practically at all patients. Most often they are shown in the form of a pain syndrome in joints or in a stomach. There are also the general weakness, slackness, fatigue. If to perform more detailed inspection and inspection of the patient, then it is possible to reveal also the accompanying pathologies in the form of a glomerulonephritis, inflammatory changes from blood, disturbance of immune system of an organism.
The hemorrhagic type is the most common form of a disease. The brightest and its peculiar manifestation met at the main most of patients is the so-called palpated purpura. It represents sites of hemorrhages in skin of various sizes in combination with its hypostasis. The centers of defeat are located in most cases on shins, on dorsums of feet. Important the fact that they can be revealed very easily not only at survey of skin, but also at its palpation as sites of hypostases at the same time are very well felt by a hand. This sign allows to distinguish hemorrhagic type of a polymorphic thermal angiitis from other types of purpuras (the diseases which are followed by hemorrhages in skin). However at the very beginning of a disease the picture is not so typical, it is shown generally in the form of emergence of a large number enough small educations as blisters. During quite short time they turn into the above described centers of defeat. Further the symptomatology of a disease can accrue considerably, at the same time along with sites of hypostases and spots of hemorrhages on skin of the patient there are bubbles of various sizes filled with blood. After their opening on skin there can be superficial defects or even deep ulcers. During a heat of a disease the general hypostasis of legs is noted. It is connected with disturbance of blood circulation and venous stagnation. The same centers in the form of spots of hemorrhages can arise also on a mucous membrane of a mouth. But it happens not always.
Anaphylactoid purpura of Shenleyna-Genokh. Is a peculiar kind of the previous form of diseases. It develops most often at children of younger age after the postponed acute respiratory disease (most often after quinsy proceeding with high fever). At this type of a purpura substantial increase of body temperature, strong pain in joints, pains in a stomach are noted. In a chair blood impurity appear. Manifestations on skin represent multiple small sites of hemorrhages.
Papulonodulyarny type of a disease. Is quite seldom met kind of pathology. On skin of the patient there are centers of defeat of inflammatory character which by the outward remind small knots of rounded shape with the smooth flattened surface. Nodes and large (to walnut meet and more) the sizes which konturirutsya indistinctly among healthy skin, have pale-pink coloring. At palpation of such nodes of the patient begins to complain of quite strong painful feelings. The defeat centers at this kind of a disease most often are located in the lower extremities, sometimes on hands, on a trunk. The patient at the same time can not feel absolutely any unpleasant feelings and not show to the doctor of complaints.
Papulonecrotic type. On the patient's skin at this disease there are small small knots of inflammatory character which do not act over the level of healthy skin or tower over it a little, having semi-spherical outlines. Important difference of this kind of pathology from other similar diseases is that on a surface of skin rashes the peeling is never noted. In the center of the centers skin begins to die off gradually therefore a peculiar scab which externally represents a small crust of black color is formed. If it is accurate to remove this scab by means of tweezers, then on its place several small bleeding sores are bared. After all skin elements begin to live and resolve, on their places there are the small sizes, as if stamped scars. The centers of defeat appear first of all, as a rule, on extensor surfaces on skin over joints of hands and legs. By outward and the main location they remind a papulonecrotic form of skin tuberculosis. Therefore in clinic such patients are subject to careful inspection for the purpose of statement of the right diagnosis and purpose of adequate therapy.
Pustular and ulcer type. The disease begins in most cases with emergence on skin of the small sizes of bubbles and pustules. As a result of it the picture of an acne disease (acne) or a folliculitis is simulated. Then the defeat centers during rather short time turn into big deep ulcers which have further tendency to accrescence in the peripheral direction. It occurs generally thanks to existence at the edges of ulcers of cyanotic color of the towering roller which is formed as a result of development of inflammatory process. The pathological centers can be located absolutely in any place of skin, however most often they strike, as well as other kinds of vasculites, integuments in shins. Also often process can take stomach skin, especially its lower half. After all ulcers heal, on skin of the patient there are flat or towering over level skin hems in the field of which the long time remains cyanotic coloring.
Necrotic - ulcer type. It is the heaviest kind of a thermal polymorphic angiitis. The disease begins quickly, suddenly, in clinics the term "fulminant beginning" in this respect is used. Further the disease becomes chronic, and its signs keep during very long time. Often the disease leads to a bystry aggravation of symptoms of the patient (up to extremely heavy) then there occurs his death. The main mechanism playing extremely important role in development of a disease is education in the vessels of blood clots struck with inflammatory process which in whole or in part block their gleam.
As a result of it are broken, and often the blood stream and delivery to fabrics of oxygen and nutrients completely stop. It is in turn a cause of death of the whole sites of skin, krovosnabzhayemy the struck vessel. Externally it looks in the form of the big sizes of a scab which has characteristic black coloring. The scab can appear on skin at once, but also very extensive spot of hemorrhage or a bubble of the big sizes can precede its emergence. In that case all way of development of the center - from primary education to a scab - is overcome in only several hours, at the same time considerable disturbance from the general condition of the patient is noted.
There are high fever, severe pains in the place of defeat, in joints, the general sharp weakness, an indisposition, exhaustion. Skin infarctions most often occupy the area of the lower extremities and buttocks. Further infectious complications join a basic disease, on skin in the place of a scab pyoinflammatory process develops that leads to a bigger aggravation of symptoms of the patient. During this period fever reaches the maximum, consciousness disturbances (come to light up to a coma). At non-rendering of medical care of the patient quickly perishes. However in some cases even at assistance in full it is not possible to cope with the developed permanent pathological shifts and disturbances of exchange processes in an organism. If the patient survives, then the scab on his body is gradually torn away. On its place there are ulcers which have very big sizes and depth, at the bottom of them there is a large amount of pus. They very slowly begin to live, will badly respond to drug treatment. At this time the condition of the patient still remains critical. Then on site ulcers there are quite big rough hems of skin.
Polymorphic type of a disease. At this type of a polymorphic thermal angiitis on skin of the same patient several various kinds of rashes are at the same time combined. Most often it is such centers of defeat which are inherent for other kinds of thermal angiites. In most cases the disease is shown in the form of emergence on skin of spots of reddening of inflammatory character, the same spots with sites of a cutaneous dropsy, multiple hemorrhages of the small sizes, the small small knots located directly on the surface of skin which are also surrounded with the site of hypostasis.
Diagnosis:
At a research of skin of the patient under a microscope it is possible to reveal swelling of walls of the struck vessels, especially their inside layer. In them big accumulation of the immune cells which got here from a blood channel as a result of inflammatory reactions is found. At the same time not only the whole cells, but also their fragments, fragments of kernels are visible (or so-called nuclear dust). Vascular walls become impregnated through with fibrin - the main blood protein responsible for its coagulability. Also very strongly their permeability therefore red blood cells - erythrocytes - go beyond vessels increases.
In places the vascular wall dies off that is well visible at microscopic examination. If such sites of necrosis and an ulcer are formed, then in this case there are always also blood clots as integrity of a vascular wall is the major factor interfering process of a thrombogenesis. At the same time in quality a peculiar defense reaction is strong growth of the escaped inside layer of a vascular wall. It can be so expressed that completely blocks a gleam of the vessel, considerably complicating a blood stream.
On walls of vessels can sometimes be formed and small knots, at the same time a microscopic picture considerably changes. There is a large number of various cells of the huge sizes.
Treatment of a polymorphic thermal angiitis:
Sanitation of the centers of an infection. Correction of endocrine and exchange disturbances. Antihistaminic drugs, vitamins (With, P, B15), calcium drugs, non-steroidal anti-inflammatory drugs (indometacin, Butadionum). At accurate communication with an infection - antibiotics. In hard cases - hemosorption, a plasma exchange, it is long corticosteroids in adequate doses with gradual cancellation. At chronic forms - quinolines (delagil on 1 tablet a day several months). Locally at spotty, papular and knotty rashes - occlusive bandages with corticosteroid ointments (флюцинар, Ftorokortum), linimentum "Dibunolum"; at necrotic and cankers - lotions skhimopsiny, Iruxolum ointment, Vishnevsky's ointment, Solkoseril ointment, 10% metiluratsilovy ointment. In hard cases the bed rest, hospitalization is necessary.
The forecast at the isolated damage of skin for life favorable, for full treatment often doubtful.
