Skin amyloidosis

Description:

Amyloidosis - disturbances of protein metabolism diverse by origin at which vnekletochno in fabrics of different bodies the glycoprotein amyloid is postponed. Distinguish a system and local amyloidosis. At the last deposits of amyloid are localized only in a certain body.

Skin amyloidosis reasons:

It is considered that the disease is caused by disturbance of transformation of proteins in the patient's organism. As a result of it in an organism in various bodies and fabrics pathological protein - amyloid is laid.

Skin amyloidosis symptoms:

Now there is a gradation according to which the disease is subdivided into 3 types:

primary system amyloidosis;
local lichenoid amyloidosis;
secondary system amyloidosis.
Primary system kind of a disease occurs among the population quite seldom. Most often develops at people of advanced age. Protein amyloid in large numbers at the same time collects in a stomach and intestines, in a gleam of vessels, in skeletal muscles, and also in skin. Such disbolism are resulted by characteristic external changes. Language of the patient can increase considerably in sizes, sometimes even by 3-4 times, becomes more dense, can even not find room in an oral cavity, his mobility is broken. Integuments of the person get pale coloring. In the field of natural folds of skin (most often in a groin, in the field of a pubis, hips, axillary hollows, etc.) there are very small sizes small knots over which skin pale. They are inclined to grouping among themselves and to merge. As a result of the last on skin the big pathological centers having more dark in comparison with healthy skin coloring and a rough surface are formed. There are disturbances in the general condition of the patient. He constantly feels weakness, pain in skeletal muscles, mobility of joints is broken. As a rule, the itch at the same time is not observed. In some cases patients along with an amyloidosis of skin have associated diseases, most often in the form of myelomas (a tumor of nervous trunks) and emergence of proteins in urine that demonstrates disturbance of protein metabolism in the patient's organism.

Primary localized amyloidosis form. This kind of a disease also call lichenoid. On skin of the patient there are multiple small knots having brownish coloring and which are closely grouped among themselves. Sometimes the plentiful peeling is defined on the surfaces of skin over small knots. In a form they conic or flat. Though small knots are also inclined to grouping, nevertheless they under no circumstances do not merge among themselves. External signs of this kind of a disease in many cases remind neurodermatitis. It is sometimes similar on red flat deprive. During a disease of the patient shows complaints to feeling of a severe itch in the field of the pathological centers. In the field of a part of the pathological centers growths as warty can appear. The most frequent locations of the pathological centers at a lichenoid form of an amyloidosis are area of shins, hips, is much more rare - persons.

Secondary system amyloidosis. Apparently from the name, this kind of a disease develops generally at the people having other skin or other diseases. Most often it is tuberculosis, malaria, leprosy, damages of skin of purulent character. Externally the disease is shown in the form of emergence on integuments of characteristic disks which rather dense to the touch, have dark pink coloring, at the same time the patient tests a severe itch.

Diagnosis:

Diagnosis of primary amyloidosis is difficult and demands use of all possible methods. The secondary amyloidosis of bodies is distinguished slightly easier as the disease capable to lead to its development is known.

There are no specific laboratory changes at an amyloidosis. However existence considerably of the raised SOE (50-70 mm/h) and anemia is characteristic of patients (in some cases the thrombocytosis, a fibrinosis are noted). Apply also special tests on amyloid (about a Congo red, and are removed by a methylene blue which normal change urine coloring whereas at patients they are fixed by amyloid with urine in the minimum quantities), and an electrophoretic research of proteins of urine.

At a heart amyloidosis on an ECG the low voltage of the QRS complex is registered. When carrying out an echocardiography the combination of the increased echogenicity (permeability) of a myocardium and a thickening of walls of auricles is noted that in 60-90% of cases indicates an amyloidosis.

In a proteinurichesky stage of an amyloidosis of kidneys the insignificant amount of protein in urine and periodic emergence of erythrocytes which sometimes is found only at an urocheras research on Kakovsky-Addis's method comes to light. Besides, in urine single cylinders (hyaline, granular, wax-like) and leukocytes are defined. In some cases the renal blood stream and filtering slightly decrease. In a nephrotic stage the amount of albumine increases, among proteins of urine globulins, especially their coarse particles prevail. High content of gamma glycoproteins and alpha lipoproteids in urine is noted. In an urocheras find a small amount of erythrocytes, cylinders and quite often leukocytes. Decrease in level of blood proteins with disturbance of their ratio (the albuminoglobulinovy coefficient decreases to 1,0 and below) is observed, among globulins also gamma fractions prevail alfa-2. The increased blood cholesterol level can be observed. Sometimes in blood moderate increase in content of creatinine, urea and residual nitrogen is found. Little changes of an eyeground can be observed. The great value in diagnosis belongs to a biopsy of kidneys. Besides, results of radiological and radio indicator methods of a research help to specify the diagnosis.

At a liver amyloidosis the laboratory research reveals characteristic proteinaceous shifts, especially alpha 2 - глобулинемию, immune disturbances, often hyperactivity of an alkaline phosphatase of serum, other functional trials are a little changed. The puncture biopsy of a liver has the greatest diagnostic value.

Treatment of an amyloidosis of skin:

Vitamin drugs in big dosages, especially are appointed And. Inside in the form of solution appoint vitamins E, groups B, PP. Solutions of anesthetics are entered into the area of the centers of defeat at strong feeling of an itch which interrupts a sleep and normal life of the patient. Various ointments which are widely presented at the pharmacological market now are applied.

Forecast:

The amyloidosis proceeds chronically for a long time. At severe forms of a disease the forecast is adverse. The best forecast concerning recovery is available at a lichenoid kind of a disease. However at any outcome it is always worth being afraid of a possibility of development of a disease again. Therefore such patients have to be a long time under observation of the dermatologist.