What Is Darier Disease?

A class of systemic diseases caused by insufficient secretion of adrenal corticosteroids. Caused by the disease of the adrenal gland is called primary hypoadrenal hypofunction. It was first described by Addison T. Therefore, it is called Addison's disease. Caused by diseases of the hypothalamus and / or pituitary are called secondary hypoadrenal insufficiency. The main manifestations are fatigue, loss of appetite, nausea, vomiting, low blood pressure, etc. In severe cases, symptoms of adrenal crisis such as shock, hyponatremia, and hypoglycemia may occur. Primary cases also have skin and mucous membrane pigmentation. It is necessary to use replacement therapy throughout life, that is, to add physiological amounts of adrenocortical hormones.

Addison's disease

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A class of systemic diseases caused by insufficient secretion of adrenal corticosteroids. Caused by the disease of the adrenal gland is called primary hypoadrenal hypofunction. It was first described by Addison T. Therefore, it is called Addison's disease. Caused by diseases of the hypothalamus and / or pituitary are called secondary hypoadrenal insufficiency. The main manifestations are fatigue, loss of appetite, nausea, vomiting, low blood pressure, etc. In severe cases, symptoms of adrenal crisis such as shock, hyponatremia, and hypoglycemia may occur. Primary cases also have skin and mucous membrane pigmentation. It is necessary to use replacement therapy throughout life, that is, to add physiological amounts of adrenocortical hormones.

Cause

The causes of primary and secondary are different.

Adrenal tuberculosis with primary hypoadrenalism. Is the most common cause, accounting for about 80%. Adrenal tuberculosis is generally the spread of systemic tuberculosis to the adrenal glands through bloodstream, and the lesions involve the adrenal cortex and medulla. Autoimmune adrenalitis. Also known as idiopathic adrenal hypofunction. Developed countries have reported that autoimmune adrenitis has accounted for 83.8% of the causes. Autoimmune adrenitis is the formation of antibodies against autoadrenal tissues in the body. The antigen-antibody binding reaction caused a large number of adrenal cells to be destroyed, and the normal cortex structure disappeared. It was replaced by a wide range of fibrous tissues, with diffuse infiltration of lymphocytes, plasma cells, and monocytes. Adrenal medulla are rarely affected and at least mild lymphocytic infiltration occurs. Autoimmune adrenalitis often coexists with other autoimmune diseases. About half of the patients have one or more other autoimmune diseases of other organs. Other causes. Rare causes include fungal infection, adrenal hemorrhage, malignant tumor metastasis or infiltration of leukemia into the adrenal glands, amyloidosis, adrenal destruction caused by certain drugs or radiation therapy, and adrenal vascular embolism. Adrenalectomy or major resection often results in lifelong adrenal insufficiency.

Secondary hypoadrenocortical function is often caused by various diseases of the pituitary and / or hypothalamus (including tumors, inflammation, hemorrhage, etc.), which cause the secretion of hypothalamus CRH and / or pituitary ACTH to decrease, leading to adrenocortical hormone Secondary insufficient secretion.

Addison's disease is often onset and concealed, and it is more common in adults, and it is less common in the elderly and young. Tuberculosis is often more men than women, while autoimmune people are more women than men. Pigmentation is often the earliest manifestation, appearing in exposed areas, such as the face, neck, back of the hand, etc., or areas that are easily stressed, such as the elbow, waist and sciatica. Oral mucous membranes, eyelids, palm prints, areola, and fingernails are also areas where pigmentation often occurs. Another important manifestation is general weakness, which begins to appear after exertion, but can still recover. After aggravation, he also feels weak at rest and unable to persist in work. Gastrointestinal symptoms include loss of appetite, nausea, vomiting, and abdominal pain. Significant weight loss. Blood pressure is usually at the lower limit of normal, in severe cases it is lower than normal, and there is orthostatic hypotension. Amenorrhea and menstrual disorders are common in female patients.

Hyperpigmentation is the excessive secretion of ACTH by the pituitary gland and its related results. ACTH is often reduced in patients with secondary hypoadrenal function, so there is no pigmentation.

The most severe case of hypoadrenal function is adrenal crisis, which can be life threatening. Clinical features are severe gastrointestinal symptoms, including nausea and vomiting, some with diarrhea and severe abdominal pain, low blood pressure, shock, dehydration, hyponatremia, hyperkalemia, azotemia, hypoglycemia, etc. Adrenal cramps often have clear causes, such as acute infections, fever, severe physical or mental trauma, and withdrawal of hormones.

treatment

Regardless of whether it is primary or secondary, replacement therapy is mainly used, that is, supplementing a physiological amount of glucocorticoids. The normal person's cortisol secretion fluctuates day and night regularly, with more secretion in the morning and less in the afternoon, about 30mg in one day and night. Therefore, alternative therapies should also mimic physiological fluctuations. Cortisone acetate can also be used. Primary people can add appropriate mineralocorticoids if necessary. In case of stress caused by surgery, infection, fever, trauma, etc., the dose of glucocorticoids must be doubled. Usually, the amount of salt should be increased, and at least 10 g of salt should be taken daily. When hormones cannot be taken orally, they should be given intramuscularly or intravenously. In the case of adrenal crisis, hydrocortisone should be infused intravenously within the first 24 hours. After the symptoms have improved significantly, the amount of hydrocortisone should be gradually reduced. At the same time, sufficient fluids should be added. Effective antibiotics should be used to prevent infection, correct electrolyte disorders, and target the causes Take effective measures. Most patients with adrenal crisis can be rescued after timely diagnosis and treatment. In the course of treatment, attention should be paid to the etiology treatment, such as active tuberculosis patients should be actively given anti-TB treatment. Hormone supplementation and antituberculosis treatment do not affect each other and can be performed simultaneously. If you are an autoimmune person, you should pay attention to whether there are other organ diseases, and if there are, you should treat them at the same time.

If the disease is properly treated, the patient can live normally and perform normal work. But it must be emphasized that life-long treatment is needed, and the drug cannot be stopped at will. In various stress situations, hormones should be increased in time. Patients with this disease and their relatives should be familiar with the characteristics of the disease. Patients should wear obvious signs indicating the disease, such as a card or wristband, indicating the name, address, and condition of the disease, so that they can be promptly transferred to the hospital in case of coma, body weight and other adrenal crises.