Addison's disease or bronze disease is a pathological lesion of the adrenal cortex. As a consequence, the secretion of adrenal hormones decreases. Addison's disease can affect both men and women. In the main risk group, people of the age group are 20-40 years old. Addison's disease is characterized as a progressive disease with a severe clinical picture.
The causes of addison's disease are pathological, including autoimmune( auto-allergy) processes that destroy the adrenal cortex( tuberculosis, syphilis, adrenal hemorrhages, bilateral primary or metastatic adrenal tumors, amyloidosis, lymphogranulomatosis, and so on).Sometimes chronic adrenal insufficiency is secondary and develops as a result of impaired functions of the hypothalamic-pituitary system( hypothalamic-pituitary insufficiency).
What is it?
Addison's disease is a rare endocrine disease, as a result of which the adrenal glands lose the ability to produce enough hormones, especially cortisol. This pathological condition was first described by British therapist Thomas Addison in his publication of 1855, entitled Constitutional and local effects of adrenal cortex diseases.
Characteristic of
This disease is characterized by the following clinical symptomatic complex: 1) asthenia and adynamia, 2) pigmentation of the skin and mucous membranes, 3) disorders of the gastrointestinal tract and 4) lowering of arterial blood pressure.
Symptoms of bronze disease
Asthenia and adynamia( physical and mental fatigue and impotence) are the earliest, permanent and important symptoms of bronze disease. Most often, the onset of the disease can not be accurately determined. Without any forerunners, the phenomena of rapid fatigue gradually develop from work, which was usually performed before without any special fatigue;there is a feeling of general weakness developing in the ordinary way of life. Feelings of rapid fatigue and general weakness, depending on the case, progress more or less rapidly, increase and lead the patient into a state of unquenchable general weakness and even complete physical impotence.
Muscle asthenia manifests itself in varying degrees of feeling of rapid fatigue and exhaustion of forces following any more or less severe physical stress: walking, physical work, in some patients in far-advanced stages of the disease, even after eating or changing the position of the body in bed. Sometimes muscle strength can be preserved, but, what is characteristic, the muscles are extremely fast fatigued and can not, as opposed to healthy ones, perform work for a long time.
To detect this characteristic muscle fatigue( muscular asthenia), it is suggested that the patient be repeatedly pressed several times by the hand of the dynamometer, and with each successive time the dynamometer will show smaller and smaller digits, correspondingly decreasing with each time muscle strength.
In addition to muscle asthenia, mental asthenia, intellectual apathy, usually develops at the same time. Because of adynamia and asthenia, the patient must first reduce his work, rest more often, and then throw it completely and go to bed. The general weakness can be so sharply expressed that the patient hardly turns in bed, hardly answers the questions, since even the slightest conversation tires him, avoids eating. The patient is usually in full consciousness. Only in the final stage of the severe form of the disease can there be mental disorders, depressive state, delirium, cramps and finally, a coma that ends with death.
Skin pigmentation( melasma) is the most important, conspicuous, usually drawing the attention of others around the symptom that characterizes the name of the disease. Due to the abundant deposition in the cells of the malpighian layer of the skin of the iron-free pigment( melanin), the skin acquires a peculiar dirty gray, brown, bronze or smoky color, sometimes resembling the color of the mulatto or negro skin. This melasma, hardly noticeable at first, can appear already during the period of asthenia, less often melasma is the first and early symptom.
Beginning on the face, the pigmentation can capture the entire surface of the skin or be localized in certain favorite places: on exposed parts of the body exposed to light( on the forehead, neck, back of the hands, interphalangeal joints, palmar folds), in places whereand a large deposition of the pigment is normally observed( on the breast nipples, scrotum, external genitalia, in the circumference of the navel and anus). Finally, the skin spots that are irritated and rubbed with folds of oneevery time, belt, garters, bandages, as well as the places of former furuncles, burns, mustard plasters. Sometimes on the face on a general dark background appear much darker spots the size of a pinhead to lentils. Along with strong pigmentation, there are patches of skin, slightly pigmented or even completely devoid of normal pigment, which stand out sharply against the background of the surrounding dark skin - the so-called vitiligo, or leucoderma.
Brown, aspidum-gray or black spots of different size and shape are often observed on mucous lips, gums, cheeks, on soft and hard skies, on the mucosa of the foreskin, glans penis and small lips. Pigmentation of the mucous membranes is a very important, almost pathognomonic sign of the eddyson disease. But it must nevertheless be noted that in rare cases, pigmented spots on mucous membranes can occur without the phenomena of eddyson disease. In some cases of rapidly developing eddyson disease, pigmentation of the skin and mucous membranes may be absent, just like in erased, incompletely symptomatic forms and in the early stages of the disease.
Digestive disorders of the gastrointestinal tract are very common. Poor appetite, unpleasant taste in the mouth, drooling, a number of dyspeptic phenomena in the form of eructations, sensations of pressure and heaviness in the epigastric region, finally, pains in the abdomen disturb the sick, appearing for no apparent reason. A common symptom is nausea and vomiting of a clear, viscous, colorless mucus, sometimes with an admixture of bile, appearing in the morning on an empty stomach, immediately after getting up from bed, resembling a morning vomiting in alcoholics. In more severe, progressive cases, vomiting occurs more often and not only on an empty stomach, but also after eating and drinking. From the intestine are observed mainly constipation, less often diarrhea, followed by periods of constipation. Sometimes diarrhea is profuse cholera-like. Secretion of gastric juice is different in different cases and stages of the disease;a certain regularity is not observed, but hypo-and achlorhydria are more often observed in far-reaching cases. Diarrhea can be either gastrogenic in the presence of Achilles, or may appear due to increased excitability of the vagus nerve with a lowered tone of the sympathetic nerve, or due to loss of influence of the adrenal glands on the sympathetic nerve.
Simultaneously with gastrointestinal disorders, and sometimes independently of them, there are pains in the lower back, in the hypochondria, in the sides, chest or extremities. These pains, sometimes acute, sometimes appearing, then permanent, aching, dull, not amplified by pressure, are not irradiating anywhere. Pain in the pit of the stomach can sometimes appear in the form of seizures, accompanied by nausea and vomiting and reminiscent of gastric crises in tabes. The appearance of acute attacks of pain in the entire abdomen can give rise to a fusion with acute peritonitis.
The disorders of the gastrointestinal tract described above can sometimes dominate the overall picture of the disease. However, forms are not uncommon when they are expressed in a more or less weak degree or even may be almost completely absent throughout the entire disease. In any case, the presence of these disorders contributes to weight loss and weakening of patients who are already in a state of adynamia and asthenia.
Lowering arterial blood pressure( arterial hypotension) is an important and frequent symptom. The maximum blood pressure is below 100-90, even falling to 60 mm, the minimum correspondingly decreases, although to a lesser extent, and the pulse pressure decreases. In rare cases, there is no hypotension or blood pressure is only slightly reduced. Arterial hypotension depends on a decrease in the tone of the nervous sympathetic system, which occurs either as a result of anatomical changes in the adrenal gland, or from a decrease in their function or from anatomical changes in the ventral plexus and sympathetic nerve nodes.
In addition to these major underlying symptoms, there are a number of changes in some organs and systems. So in the blood, in most cases, a number of abnormalities are noted. Usually, moderate hypochromic anemia is observed. With a normal number of leukocytes, lymphocytosis with neutropenia usually occurs;less often eosinophilia and monocytosis. Speaking of lymphocytosis, it should be noted that it is often observed the so-called status thymico-lymphaticus. The number of blood platelets, the duration of bleeding time, blood coagulation do not give special deviations from the norm. There is no parallel between the severity of the disease and the morphological picture of the blood.
In most cases, a reduced content of blood sugar in fasting. Accordingly, the sugar curve after the end of the glucose load or after intramuscular injection of 1-2 mg of epinephrine yields not so great a rise as in healthy individuals, the curve falls not after 2 hours, but much later, and there is no fall below the initial figure. In relation to carbohydrates, increased endurance is noted;sugar is not found in the urine, either after a heavy load of carbohydrates, or after intramuscular injection, even 2 mg of epinephrine.
From the side of the cardiovascular system, in addition to the already indicated arterial hypotension, there is a small, weak filling and tension, a rhythmic, usually frequent pulse. The heart and the aorta are often hypoplastic. Inorganic systolic noises are heard due to anemia and altered nutrition of the heart muscle. Patients complain of a number of abnormal unpleasant sensations, in the form of palpitations, dyspnoea with movements and slightest physical strains.
In the lungs, a tubercular process of various degrees of development and compensation is often observed. The usual urine test does not give deviations from the norm, but often there is a decrease in the concentration ability of the kidneys and a decrease in the release of water under the water load. The function of the sexual glands in clearly defined cases is almost always lowered: in men - the decrease of libido and the weakening of potency;in women - often amenorrhea;conception is rare and pregnancy is often interrupted prematurely
From the side of the neuro-psychic sphere, in addition to adynamia and asthenia, in the beginning there may be increased excitability, irritability, mood variability, but soon with the progression of the disease this is replaced by increased fatigue, decline of forces indicated above as basic symptoms by adynamicsand asthenia, lack of energy, indecisiveness, apathy, indifference and depressed state to a complete stupor. Dizziness right up to fainting is not uncommon. In rare cases, in the final stage there are delusions, convulsions and coma.
Patients complain of chilliness. The temperature is normal or even lower, if there is no active process in the lungs or associated infections.
How Addison's disease looks like: detailed photos of
The photo shows how the part of the hand looks like in Addison's disease( bronze disease):
Skin pigmentation with addison's disease Forms of eddyson disease
If there are obvious cardinal symptoms, then this is a clearly expressed typical formdisease. However, there are often incomplete, erased forms of the disease( formes frustes), where there are one or two major symptoms, and then the disease presents great difficulties for recognition.
Distinguish: 1) asthenic, 2) gastrointestinal, 3) melanodermic, 4) painful forms. The so-called false-peritonitic form should be referred to the latter, with sudden strong pains in the abdomen, persistent constipation, vomiting, retracted or swollen abdomen, general depression and growing heart weakness leading to death.
Unclear, worn-out forms include those states of adynamia, asthenia and hypotension that occur without melanoderma, more or less chronically, in the basis of which there are sometimes no anatomical changes in the adrenal glands and which are considered as states of adrenal hypofunction and sympathetic system.
In children, the Eddyson's disease is characterized by a strong pigmentation, diarrhea, marked neuropsychic phenomena and a rapid course ending in death. In the elderly, strong asthenia, adynamia, apathy, drowsiness come to the fore;death occurs when cachexia occurs. Pigmentation, on the contrary, is mild.
Diagnosis of
When recognizing the disease in the presence of melasma, one must bear in mind all other physiological and pathological conditions in which the appearance of a similar pigment is also observed.
So, it is necessary to remember about the intensified pigmentation at pregnancy, chronic diseases of a uterus and ovaries;about sunburn, spreading to all places exposed to the action of radiant energy( sun, ultraviolet rays of a quartz lamp, X-rays);about the pigmentation on the skin of vagabonds and people who rarely wash, do not change clothes, who suffer from lice;about bronze cirrhosis of the liver with or without diabetes, in which there is an increase in the liver, spleen and often glycosuria;about the so-called biliary melasma in some liver patients, especially those suffering from chronic obstructive jaundice with pancreas or faker papilla gland cancer;about pigmentation in case of Gaucher disease( splenomegaly, heredity and family character of the disease), about pellagra, about grape disease, about various kinds of cachexes( for tuberculosis, cancer, pernicious anemia), usually without pigmentation of mucous membranes;finally, on arsenic melasma.
Diagnosis of the disease in the early stages in the absence of pigmentation is always difficult, since asthenia and gastrointestinal disorders can be observed in diseases that have nothing to do with the eddyson symptom complex. Careful study of other diseases, observation of patients, progression of the disease, the appearance of other major symptoms and especially the pigmentation of the skin and mucous membranes confirm the diagnosis.
When recognizing oddsymptomnyh incomplete forms of the disease in the absence of melasma, you should use: 1) a test for provocative pigmentation( at the place of the delivered fly or barley, more or less severe pigmentation develops);2) dynamometric determination of muscle fatigue;3) definitely the dynamics of the sugar curve in the blood before and after the end of the load with glucose or intramuscular injection of 1-2 mg of epinephrine;4) a test for increased endurance to carbohydrates;5) lymphocytosis, monocytosis and frequent hypereosinophilia in the blood;6) signs of status thymico-lymphaticus;7) increased endurance tolerance to the extracts of the thyroid gland and the posterior lobe of the pituitary gland.
Etiology
As the etiological factors of the disease, tuberculosis of the adrenal gland should be put first;then follow the congenital absence or hypoplasia of the adrenal glands, infections: syphilis, diphtheria, typhus, influenza, various neoplasms and destructive processes and the type of hemorrhage, sclerosis and degeneration of the adrenal glands.
Injuries, wounds, air concussions, intestinal infections and even mental shocks are noted as etiological factors in the development of both clear and erased forms of bronze disease and the so-called symptoms of benign functional adrenal insufficiency or the phenomena of eddysonism.
Pathological anatomy
When microscopic examination of pigmented areas of the skin and mucous membranes, excessive deposition of grains of brown-black melanin( iron-free) pigment in the cells of the malpighian layer and the connective tissue part of the skin is detected.
In 70% of cases, tuberculosis is found in various stages, almost completely destroyed by the adrenal glands. Often, tuberculosis granuloma affects the adjacent areas of the nervous sympathetic system and the solar plexus. Thus, the process that destroys the adrenal glands very often produces changes in the important parts of the ventral part of the sympathetic nerve. Only rarely tuberculosis of the adrenal gland is the only active focus. More often there is a tuberculosis lesion in other organs, especially in the lungs.
In the absence of tubercular lesion, the following were found: congenital absence, aplasia or hypoplasia of the adrenal glands, hypoplasia and atrophy of adrenal medulla and all chromophilic tissue, hypoplasia or cirrhotic adrenal degeneration due to diffuse syphilitic processes, gum and after acute infections, amyloidosis, malignant neoplasm, cystic degeneration, cavernous angioma, hemorrhages and hematomas, thrombosis of the veins, embolism of the vessels of the adrenal glands, necrosis, suppuration and even einokokk.
In the vast majority of cases, along with those or other adrenal lesions, changes are observed in the abdominal sympathetic nodules and nerves( degeneration and pigmentation of nerve cells, semilunar nodes, sclerosis and degeneration of the nervous tissue, hypoplasia, destruction and disappearance of the chromaffin tissue scattered along the sympathetic nerve tract).
In very rare cases, no changes have been found in either the adrenal or sympathetic nervous system. Sometimes they found thymus persistens, hyperplasia of the lymph glands, tonsils and lymphatic tissue at the root of the tongue.
The course and prognosis of
The course and prognosis of bronze disease is very diverse depending on the etiological factors, the nature of the underlying disease, the incidence of progression or remission and recovery, and of the associated diseases.
Therefore, there are observed as acute cases, ending in death in a few days, and subacute, in which death occurs after 6-12 months. Finally, cases of chronic benign course are far from rare, lasting for many years with spontaneous process stops and relapses. Cases of recovery are also described, especially with syphilitic etiology and reversible processes in the adrenal glands or sympathetic nervous system underlying the emergence of the eddysonian symptom complex in the so-called benign functional adrenal insufficiency.
It should be remembered that eddysonics are not very resistant to many harmful effects, such as physical fatigue, mental shock, trauma, acute infections and various intercurrent diseases, all of which can worsen the course of the disease, remove the patient from equilibrium and accelerate the fatal end.
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