Addison's disease or bronze disease is a pathological lesion of the adrenal cortex. As a consequence, the secretion of adrenal hormones is reduced. Addison's disease can affect both men and women. In the main risk group, people of the age group 20-40 years. Addison's disease is characterized as a progressive disease with a severe clinical picture.
The causes of Addison's disease are pathological, including autoimmune (autoallergy), processes that destroy the adrenal cortex (tuberculosis, syphilis, adrenal hemorrhage, bilateral primary or metastatic adrenal tumors, amyloidosis, lymphogranulomatosis, etc.). Sometimes chronic adrenal insufficiency is secondary and develops as a result of impaired function of the hypothalamic-pituitary system (hypothalamic-pituitary insufficiency).
What it is?
Addison's disease is a rare endocrine disease, as a result of which the adrenal glands lose their ability to produce enough hormones, primarily cortisol. This pathological condition was first described by the British therapist Thomas Addison in his 1855 publication entitled The Constitutional and Local Consequences of Diseases of the Adrenal Cortex.
This disease is characterized by the following clinical symptom complex: 1) asthenia and adynamia, 2) pigmentation of the skin and mucous membranes, 3) disorders of the gastrointestinal tract, and 4) Decrease in arterial blood pressure.
Symptoms of bronze disease
Asthenia and weakness (physical and mental fatigue and impotence) are the earliest, most persistent and important symptoms of bronze disease. Most often, the onset of the disease cannot be accurately determined. Without any precursors, the phenomena of quick fatigue from work, usually performed before without much fatigue, gradually develop; there is a feeling of general weakness that develops in a normal way of life. Sensations of rapid fatigability and general weakness, depending on the case, more or less rapidly progress, intensify and lead the patient to a state of intractable general weakness and even complete physical impotence.
Muscular asthenia is expressed in varying degrees of feeling of rapid fatigue and exhaustion of forces occurring after any more or less strong physical exertion: walking, physical work, in some patients in advanced stages of the disease even after eating or changing the position of the body in bed. Sometimes muscle strength can be maintained, but, characteristically, muscles tire extremely quickly and cannot, in contrast to healthy ones, perform work for a long time.
To identify this characteristic muscle fatigue (muscular asthenia), it is suggested that the patient should repeatedly squeeze the dynamometer with his hand, and with each successive time the dynamometer will show smaller and smaller numbers, respectively, the muscular strength decreasing each time.
In addition to muscle asthenia, mental asthenia and intellectual apathy usually develop simultaneously. Because of adynamia and asthenia, the patient is forced to first reduce his work, rest more often, and then throw it altogether and lie down in bed. General weakness can be so sharply expressed that the patient barely turns in bed, barely answers questions, as even the slightest conversation tires him, avoids food. The patient is usually fully conscious. Only in the final stage of a severe form of the disease can there be mental disorders, depression, delirium, convulsions, and finally, a coma that ends in death.
Pigmentation of the skin (melasma) is the most important, eye-catching, usually noticeable symptom surrounding the name of the disease. Due to the abundant deposition of iron-free pigment (melanin) in the malpighian layer of the skin, the skin acquires a peculiar dirty-gray, brown, bronze or smoky color, sometimes resembling the skin of a mulatto or black. This melasma, barely noticeable at the beginning, may appear already during the period of asthenia, less often melasma is the first and early symptom.
Starting on the face, pigmentation can capture the entire surface of the skin or be localized on individual favorite places: on exposed parts of the body exposed to light (on the forehead, neck, dorsum of the hands, interphalangeal joints, on the palmar folds), on the places where in normal, there is a large deposition of pigment (on the breast nipples, scrotum, external genital organs, around the navel and the anus), finally, skin areas that are exposed to irritation and rubbing with folds of clothing turn out to be particularly pigmented, belt, garters, bandages, as well as places of former boils, burns, mustard plaster. Sometimes much darker spots, ranging in size from a pinhead to lentils, appear on the face against a general dark background. Along with strong pigmentation, there are areas of skin that are poorly 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.
Often there is the appearance of brown, gray-gray or black spots of various sizes and shapes on the mucous lips, gums, cheeks, on the soft and hard palate, on the mucous membrane of the foreskin, glans penis and small lips. Pigmentation of mucous membranes is a very important, almost pathognomonic sign of Eddison disease. But we must still note that in rare cases pigment spots on the mucous membranes can occur without the occurrence of Eddison disease. In some cases of rapidly developing Eddisonov disease, the pigmentation of the skin and mucous membranes may be absent, just as in erased, non-posymptomatic forms and in the early stages of the disease.
Disorders of the gastrointestinal tract are very common. Bad appetite, unpleasant taste in the mouth, drooling, a number of dyspeptic symptoms such as belching, pressure and heaviness in the epigastric region, and finally, pain in the abdomen bother patients, appearing for no apparent reason. A common symptom is nausea and vomiting of transparent, viscous, colorless mucus, sometimes with an admixture of bile, appearing in the morning on an empty stomach, immediately after getting out of bed, resembling morning vomiting in alcoholics. In more severe, progressive cases, vomiting is more common and not only on an empty stomach, but also after eating and drinking. On the part of the intestine are mainly constipation, less diarrhea, alternating periods of constipation. Sometimes diarrhea are profuse cholera-like character. The secretion of gastric juice is different in different cases and stages of the disease; a certain regularity is not observed, but in far advanced cases hypo- and achlorhydria is more often observed. Diarrheas can be either gastrogenic in the presence of ahilia, or they can appear due to increased excitability of the vagus nerve with a reduced tone of the sympathetic nerve, or due to the loss of the 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 hypochondria, sides, chest or limbs. These pains, then sharp, sometimes appearing, then constant, aching, dull, not aggravated by pressure, not radiating anywhere. Pain in the stomach can sometimes appear in the form of seizures, accompanied by nausea and vomiting and resemble gastric crises during taves. The appearance of acute bouts of pain in the entire abdomen may give rise to merging 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 may even be almost completely absent throughout the course of the disease. In any case, the presence of these disorders contributes to weight loss and weakening of patients, who are already in the state of adynamia and asthenia.
A decrease in 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 decreases accordingly, albeit to a lesser extent, and the pulse pressure decreases. In rare cases, hypotension is not observed or blood pressure is only slightly reduced. Hypotension depends on a decrease in the tone of the nervous sympathetic system, either due to anatomical changes in the adrenal glands, or from a decrease in their function, or from anatomical changes in the abdominal plexuses and sympathetic nerve nodes.
In addition to these main symptoms, a number of changes in certain organs and systems should be indicated. So in the blood, in most cases, a number of deviations from the norm are noted. Mild anemia of the hypochromic type is usually observed. With a normal leukocyte count, lymphocytosis most often occurs with neutropenia; less eosinophilia and monocytosis. Speaking about lymphocytosis, it should be noted that the so-called status thymico-lymphaticus is often observed. The number of platelets, the duration of bleeding time, blood clotting do not give special deviations from the norm. There is no parallelism between the severity of the disease and the morphological picture of the blood.
In most cases, find a low blood sugar fasting. Corresponding to hypoglycemia, the sugar curve at the end of glucose loading or after intramuscular injection, 1-2 mg of adrenaline gives a not-so-great rise as in healthy ones, the curve does not fall after 2 hours, but much later, and there is no drop below the original figure. In relation to carbohydrates, increased endurance is noted; sugar is not detected in the urine either after a large load of carbohydrates, or after intramuscular injection, even 2 mg of adrenaline.
On the part of the cardiovascular system, in addition to the already indicated arterial hypotension, there is a small, weak filling and tension, a rhythmic, usually rapid pulse. The heart and aorta are often hypoplastic. Inorganic systolic sounds due to anemia and altered cardiac muscle are heard. Patients complain of a number of abnormal unpleasant sensations, in the form of palpitations, shortness of breath during movements and the slightest physical stresses.
The lung often has a tuberculous process of varying degrees of development and compensation. Routine urine testing does not deviate from the norm, but often there is a decrease in the concentration ability of the kidneys and a decrease in the excretion of water under water load. The function of the sex glands in clearly expressed cases is almost always reduced: in men, a decrease in the libido and a weakening of the potency; in women, often amenorrhea; conception occurs rarely and pregnancy often terminates prematurely
On the part of the neuropsychic sphere, in addition to adynamia and asthenia, first anxiety, irritability, mood variability can be observed, but soon with the progression of the disease this is replaced by increased fatigue, loss of strength, indicated above as the main symptoms of adynamy and asthenia, lack of energy, indecision, apathy , indifference and depressive state to a complete stupor. Dizziness up to fainting is not uncommon. In rare cases, in the final stage, there are delirium, convulsions and coma.
Patients complain of coldness. The temperature is normal or even lowered if there is no active process in the lungs or associated infections.
How Addison's Disease Looks: Detailed Photos
The photo shows how a part of the hand looks like with Addison's disease (bronze):Pigmentation of the skin in Addison's disease
Forms of eddisonovoy disease
If there are major cardinal symptoms, this is a clearly expressed typical form of the disease. However, there are often incomplete, erased forms of the disease (formes frustes), where there are one or two main symptoms, and then the disease presents great difficulties for recognition.
Distinguish: 1) asthenic, 2) gastrointestinal, 3) melasma-thermal, 4) painful forms. The latter should include the so-called pseudoperitonitis form, in which the sudden emergence of severe abdominal pain, persistent constipation, vomiting, retracted or distended abdomen, general depression and increasing heart weakness leading to death come to the fore.
The obscure, erased forms should also include those states of adynamia, asthenia and hypotension, occurring without melanoderma, more or less chronically, sometimes based on anatomical changes of the adrenal glands and which are considered as states of adrenal hypofunction and sympathetic system.
In children, Eddisonov disease is characterized by strong pigmentation, diarrhea, pronounced neuropsychiatric phenomena and a rapid course, ending in death. In old age, severe asthenia, weakness, apathy, drowsiness; death occurs with symptoms of cachexia. Pigmentation, on the contrary, is mild.
When recognizing a disease in the presence of melasma, all other physiological and pathological conditions must be kept in mind, in which the appearance of a similar pigment is also observed.
So, we must remember about the enhanced pigmentation during pregnancy, chronic diseases of the uterus and ovaries; tanning that extends to all places exposed to radiant energy (the sun, ultraviolet rays of a quartz lamp, x-rays); pigmentation on the skin of vagrants and people who rarely wash themselves, do not change clothes, and suffer from lice; about bronze cirrhosis of the liver with or without diabetes mellitus, in which there is an enlargement of the liver, spleen, and often glycosuria; about the so-called biliary melasma in some liver patients, especially those suffering from chronic obstructive jaundice in cancer of the head of the pancreas or Vater papilla gland; on pigmentation in Gaucher disease (splenomegaly, heredity and familial nature of the disease), on pellagra, on the basis of the disease, on various kinds of cachexia (for tuberculosis, cancer, pernicious anemia) usually without mucous membrane pigmentation; Finally, about arsenic melasma.
The diagnosis of the disease in the early stages in the absence of pigmentation is always difficult, since asthenia and gastrointestinal disorders can also be observed in diseases that have nothing to do with the Eddison Symptom Complex. Careful examination of other diseases, observation of patients, the 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 one-asymptomatic incomplete forms of the disease in the absence of melasma, the following should be used: 1) a test for provocative pigmentation (in place of the delivered fly or mustard plaster, more or less strong 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 glucose loading or intramuscular injection of 1-2 mg of adrenaline; 4) test pas increased endurance to carbohydrates; 5) lymphocytosis, monocytosis and frequent giperosinophilia in the blood; 6) signs of status thymico-lymphaticus; 7) Increased addison endurance to the extracts of the thyroid gland and the posterior lobe of the pituitary gland.
As the etiological moments of the disease, adrenal tuberculosis should be put in the first place; then follow the congenital absence or hypoplasia of the adrenal glands, infections: syphilis, diphtheria, typhus, influenza, various neoplasms and destructive processes and the form of hemorrhages, sclerosis and degeneration of the adrenal glands.
Injuries, injuries, air contusions, intestinal infections and even mental disturbances are noted as etiological moments in the development of both clear and erased forms of bronze disease and the so-called symptoms of benign adrenal functional insufficiency or phenomena of addisonism.
Microscopic examination of pigmented areas of the skin and mucous membranes reveal excessive deposition of grains of brown and black pigment melanin (not containing iron) in the cells of the malpighian layer and the connective tissue part of the skin.
In 70% of cases, those affected by tuberculosis are found in different stages, almost completely destroyed by the adrenal glands. Often tuberculosis granuloma affects the adjacent areas of the sympathetic nervous system and the solar plexus. Thus, the process that destroys the adrenal glands very often produces changes in important parts of the abdominal part of the sympathetic nerve. Only rarely is adrenal tuberculosis the only active focus. More often there is a tuberculous lesion in other organs, especially in the lungs.
In the absence of tuberculous lesions were observed: congenital absence, aplasia or hypoplasia adrenal hypoplasia and atrophy of the adrenal medulla and the whole chromaffin tissue, hypoplasia or cirrhotic adrenal degeneration due to diffuse syphilitic processes gummas and after acute infections, amyloidosis, lesion malignancy, cystic degeneration, cavernous angioma, hemorrhages and hematomas, vein thrombosis, adrenal embolism, necrosis, suppuration and even echinoca kk.
In the vast majority of cases, along with these or other lesions of the adrenal glands, changes in the abdominal sympathetic glands and nerves are observed (regeneration and pigmentation of nerve cells, semilunar nodes, sclerosis and degeneration of the nervous tissue, hypoplasia, destruction and disappearance of chromaphin tissue scattered throughout the sympathetic nerve tract) .
In very rare cases, no changes were found in either the adrenal glands or the sympathetic nervous system. Sometimes found thymus persistens, hyperplasia of the lymphatic glands, tonsils and lymphatic tissue at the root of the tongue.
Current and forecast
The course and prognosis of a bronze disease is very diverse, depending on the etiological moments, on the nature of the underlying disease, on the propensity to progress or subside and recover, and on the diseases that have joined.
Therefore, there are both acute cases ending in death in a few days, and subacute cases in which death occurs after 6-12 months. Finally, cases of chronic benign course that have lasted for many years with spontaneous process stops and relapses are far from rare. Cases of recovery are also described, especially in case of syphilitic etiology and reversible processes in the adrenal glands or the sympathetic nervous system, which are the basis for the appearance of the Eddison Symptom Complex in the so-called benign functional insufficiency of the adrenal glands.
We must remember that addisoni are unstable with respect to many harmful effects, such as: physical exhaustion, mental shock, injuries, acute infections and various intercurrent diseases - all this can worsen the course of the disease, bring the patient out of balance and accelerate the fatal end.