Enteropatichesky acrodermatitis

Description:

The Enteropatichesky acrodermatitis described by 1935-1942 is the general disease which is observed most often aged from the first weeks up to 1,5 years of life. Sometimes occurs at children of more advanced age and at adults. Quite often found wrong diagnosis of a disease which is taken for eczema, candidiasis, a pyoderma, dermatitis leads to untimely and inadequate therapy of children of chest age and sharp deterioration in the general condition of the child up to a lethal outcome.

Symptoms of the Enteropatichesky acrodermatitis:

Emergence of rash around all natural foramens, and also in buttocks, inguinofemoral folds, generative organs, on distal departments of extremities is characteristic. There are hyperemic edematous centers with vezikulo-violent and pustular elements on a surface. Rashes gradually extend symmetrically to elbow, knee joints, a trunk. Bubbles turn into serous and purulent crusts on which places difficult healing erosion and ulcers of red color with a cyanotic shade form. Nikolsky's symptom is negative. At the same time mucous membranes (often candidosis character) in the form of stomatitis, a cheilitis, a blefarokonjyunktivit, a vulvovaginitis, a balanoposthitis are surprised, there are paronychias and onychias, sometimes with the phenomena of a hyponychial hyperkeratosis and an onikholizis. Quickly enough develops partial, and the total alopecia is more often, up to loss of eyelashes and eyebrows. The photophobia is expressed, sometimes there is an ectropion a century.

Along with damage of skin and mucous membranes, and sometimes before their emergence there are gastrointestinal frustration in the form of frequent (from 3 to 30 times a day) liquid, greenish, mucous, with a putrefactive smell of the chair containing the undigested remains of food. The meteorism, concern of the child are noted. The disturbance of intestinal absorption arising in connection with the dispepsichesky phenomena quickly enough results in deficit of body weight and a growth inhibition, to emergence of heavy dystrophy. The general condition of the patient sharply worsens, being followed by the subfebrile temperature or fever of the wrong type. Anorexia, slackness, sleeplessness, irritability, tearfulness develop that is caused by disturbance of mentality of the child.

The disease has the long, chronic, progressing current, and at irrational therapy often recurs. The Enteropatichesky acrodermatitis, as a rule, is complicated by consecutive infection in the form of S.'s accession of albicans, staphylococcus and a streptococcus, vulgar a protea and a pyocyanic stick who are revealed in crops from the centers on skin, mucous membranes of a mouth, a nose, eyes, generative organs, from separated ears, in Calais, blood. At most of patients changes of visceral bodies in the form of bronchitis, pneumonia, a SARS, gepatokholangit are noted. In blood find hypochromia anemia, increase in SOE, a leukocytosis, reduction of content of crude protein, a disproteinemia, decrease in level of cholesterol, an alkaline phosphatase, calcium, phosphorus.

Reasons of the Enteropatichesky acrodermatitis:

The disease is the genodermatosis which is transferred autosomal рецессивно. Quite often arises at children from marriages between blood relatives. Major importance in a pathogeny has the deficit of zinc in an organism arising because of genetically caused enzymopathy. Histochemical researches of a bioptat of a mucous membrane of a fenny gut found low activity of a succinatedehydrogenase and leucineaminopeptidase.

It is known that absorption of zinc is carried out in a duodenum by means of a tsinkosvyazyvayushchy factor (TsSF) which is low-molecular protein which is secreted by a pancreas. At an enteropatichesky acrodermatitis at the newborn the gene controlling activity of TsSF Defective in this connection there is zinc malabsorption. TsSF is available in maternal milk and is absent in cow. Therefore developing of a disease or its progressing current happens usually at transfer of the child from natural feeding on artificial. Owing to TsSF of a gipotsinkemiya arising because of insufficiency tryptophane exchange is broken, synthesis of a number of amino acids decreases, there is oversaturation of cells fatty acids, triglycerides, tension of cellular and humoral immunity decreases (the IGA and IGM levels especially decrease). The ratio of linoleic and olein acids breaking functional activity of epidermis changes and clinical manifestations of an enteropatichesky acrodermatitis develop: It is noted that deficit of zinc leads to a sharp dissonance between phagocytosis, bactericidal activity and a chemotaxis of neutrophils.

Treatment of the Enteropatichesky acrodermatitis:

As replacement therapy inside appoint it is long for a number of years, zinc drugs in the form of sulfate, a gluconate or its oxide. Best of all children of chest age transfer a zinc oxide in a dose of 0,04-0,05 3 times to days (100-120 mg of zinc). In 7-10 days after an initiation of treatment changes on skin, mucous membranes and intestinal frustration die away, growth of hair and nails begins, body weight increases. After disappearance of displays of a disease the daily dose gradually decreases to 50 mg which is supporting in further life of the child. Children have to receive natural feeding by women's milk.

Replacement therapy by drugs of zinc is combined with the complex treatment including antibacterial drugs: antibiotics of a broad spectrum of activity - at complications a piokokkovy infection and nystatin, levorinum and other antifungalny drugs - at stratification of candidiasis. Also excitants - gamma-globulin, albumine, vitamins A, With, a complex B are appointed. For improvement of digestion use fermental drugs - a gastric juice, Pancreatinum, festal, and at dysbacteriosis - лактобактерин or bifidumbacterium.

At a severe disease glucocorticoid hormones inside or parenterally on 1 mg/kg are necessary (counting on Prednisolonum).

Applied (from the 50th until the end of the 70th years) for treatment of patients with an enteropatichesky acrodermatitis haloid derivatives of oxyquinoline (Enteroseptolum, Intestopanum) with use of also supporting dosages were the most effective remedies of treatment until as began to use zinc drugs. Now derivatives of oxyquinoline do not apply as they can cause side effects in the form of peripheral neuritis, damages of a retina and optic nerve.

Outwardly the aqueous solutions of aniline dyes, ointments containing levorinum, 2-3% of Naphthalanum, bismuth, Ichthyolum, 1% канестен apply 1%.