ERITRODERMY Ya (erythrodermia; Greek erythros red + derma skin) — the reddening of all skin (a universal erythrosis) or its extensive sites (a partial erythrosis) which is followed by puffiness, infiltration and a peeling. The term «erythrosis» is used also for designation of a number of nosological forms.
AA. develops on previously not changed skin (primary E.) or arises in the course of a current of any dermatosis (secondary E.). Besides, distinguish a nonspecific and specific erythrosis. Nonspecific E. represents universal reaction of skin to nek-ry pharmaceuticals and toxicants (see Toxidermias) or the wedge, a kind of a nek-ry dermatosis is at their adverse current (see Deverzhi a disease, Psoriasis). Specific E. has the tumoral nature and represents lymphoma of skin. Carry Wilson's erythrosis to them — Brock (generalized subacute exfoliative dermatitis), AA. at a reticulosis of skin (see) and an erythrosis of Al-lopo — Benye, being an erythrodermic form of fungoid mycosis (see Mycosis fungoid). The reason zlokachestvenno proceeding specific E. the proliferation of unripe lymphocytes which is initially arising in skin serves. Existence specific E. at malignant diseases of blood — leukoses (see), and also a lymphogranulomatosis (see) it is called by nek-ry researchers into question. Development E., irrespective of the reason which caused it, connect with disturbance of immunity. Row E. represents independent nosological forms, napr, a desquamative erythrosis of Leyner, an ikhtioziformny inborn erythrosis (see the Ichthyosis).
In article forms E are described., not covered in the edition with independent articles.
Wilson's erythrosis — Brock. To this form E. precede it is long the existing changes of skin reminding displays of eczema (see), an eczematid or neurodermatitis (see). The bright hyperemia and the expressed puffiness of skin, a macrolaminar peeling with an arrangement of scales parallel ranks are characteristic of the created disease; palmar and bottom keratosis, hair loss and nails; increase limf, nodes; the expressed itch and oznoba. In blood the leukocytosis, a monocytosis, an eosinophilia, acceleration of ROE is noted.
At E. in a derma polymorphocellular infiltration (among cells find unripe lymphocytes) is observed; in limf, nodes — a picture of a dermatopatichesky adenitis (see the Lipomelanotichesky reticulosis).
The diagnosis is complicated. It is established on the basis by a wedge, pictures, results of repeated biopsies of skin and limf, nodes. The differential diagnosis is carried out with other forms E.
Treatment is carried out by glucocorticosteroid hormones to combinations with cytostatics. The disease can proceed many years, the forecast for life favorable.
The desquamative erit-rodermiyaleynera, or Leyner's disease — A. Moussous, is for the first time allocated in 1905 by the fr. pediatrician A. Moussous. In 1907 it is in details described avstr. the pediatrician Leyner (To. Leiner), observing a wedge, a picture and disease at 43 children.
The etiology is not established. Assume that deficit of a complex of vitamins of group B is the cornerstone of a disease. In developing of a disease also the vitamin deficiency of A, E, C matters. As the sensibilizing factor the large role is played by a pneumococcal and candidosis infection. Considerable decrease in content of biotin (see) in maternal milk, blood and urine of children with a desquamative erythrosis of Leyner is revealed. The proof of value of a hypovitaminosis in genesis of a disease is obtaining good therapeutic effect at purpose of biotin, B12 vitamin, folic to - you. Children have messages on insufficiency with a desquamative erythrosis of Leyner of the opsonic phagocytal activity caused by deficit of a component of Saturday of a serumal complement (see Opsonins). This deficit can be caused genetically since family cases were observed.
At a vitamin deficiency disbolism is noted. Deficit of vitamin E and the lowered function of a pancreas are followed by disturbance of absorption of fats (see Malabsorption a syndrome).
The disease usually begins in the period of a neonatality (more rare aged is more senior 1 month, but no later than 3-month age). In the beginning skin in buttocks and inguinal folds becomes hyperemic and infiltrirovanny, reminds an intertrigo. However the therapy applied at treatment of an intertrigo does not give effect. In the next days and weeks the erythema extends to skin of a trunk, the person, axillary hollows; in affected areas skin infiltrutsya, is shelled (fig.). On a pilar part of the head accumulations of the fat scales of yellow-gray color which are going down on area of a forehead to eyebrows are formed. The child's face takes a masklike form. In the period of developed a wedge, pictures of a disease are noted puffiness, a hyperemia, ma-
by Fig. The newborn with a desquamative erythrosis of Leyner: a widespread erythema with a large lamellar peeling on skin of a trunk, the person, a pilar part of the head.
tseration and skin moknuty pleated (inguinal, buttock). The deep, hardly healing cracks are pleated formed. Dystrophy of nails in the form of cross striation is possible.
Children are uneasy, sleep badly. The general serious condition that is caused not only by damage of skin, but also the heavy dispeptic frustration (vomiting, kcal liquid with greens, impurity of slime and lumps of undigested food, a chair
from 5 to 10 times a day) connected with defeat of a small bowel, and also a hypotrophy, anemia and a hypoalbuminemia. Often hypostases come to light (especially on the lower extremities). Mac-Klyura's test — Aldrich (see Mac-Klyura — Aldrich test) is reduced (on average up to 10,3 min.).
The expressed hypochromia anemia is noted: hemoglobin from
6,0 8% (60 g/l) to 11,5 g of % (115 g/l) at norm of 14,2 — 17,0 g of % (
142 — 170 g! k), erythrocytes from 1,9 million in 1 mkl (1,9-1012/d) to 3 million in 1 mkl (3-1012/l) at norm of 4,5 — 5,3 million in 1 mkl (4,5 — 5,3 * 1012/l); at most of children — in blood small increase in number of leukocytes (including eosinophils to 5 — 7%), is frequent acceleration of ROE. Content of chlorides is increased; blood protein from 37 to 50 g/l with a disproteinemia due to decrease in content of albumine and increase ah - and a2-globu-linov; the amount of cholesterol is reduced; several hyperactivity of zymohexase (9 — 12 units).
The disease can be complicated by otitis, pneumonia, multiple abscesses, phlegmons, lymphadenitis, purulent conjunctivitis, pyelonephritis that leads to a serious toksiko-septic condition. From blood of such patients golden staphylococcus, as a rule, slabochuvstvitelny or insensitive to various antibiotics is sowed.
The diagnosis is established on the basis by a wedge, pictures. Differentiate a disease with an ichthyosis (see) and candidiasis (see); differential diagnosis with exfoliative dermatitis of Ritter is especially difficult, at Krom erosive sites of skin and amotio of epidermis are noted (see Ritter dermatitis exfoliative).
Appoint bathtubs with broth of a camomile; skin is oiled luccu or peach-kernel, disinfecting, keratoplastichesky means; the becoming wet sites, especially folds of skin, process aniline dyes (e.g., 1% water solution of methylene blue). Apply prednizolonovy cream in mix with children's cream on the inflamed sites of skin (1:3; 1:4) or naf-talanovy cream. Appoint a complex of polyneuramins, and also ascorbic to - that. For normalization of activity went. - kish. a path apply enzymes (pepsin, Pancreatinum); at dysbacteriosis — laktobakterin, bifidumbacterium, bifikol, etc. In case of a toksiko-septic state appoint antibiotics of a broad spectrum of activity (tseporin, ampioks, lincomycin, gentamycin). For increase immunol. reactivity use gamma-globulin, polyglobulin, intravenous drop injection of 5 — 10% of solution of glucose with Ringer's solution — Locke, Haemodesum, reopoliglyukin, 5% solution of albumine; transfusions of blood or plasma. In some cases at a serious toksiko-septic condition glucocorticoids are shown.
The forecast at timely treatment favorable; at a heavy current, a toksiko-septic state and sepsis the lethal outcome is possible.
For the prevention at the child of a desquamative erythrosis Leyner the woman during pregnancy shall receive rather caloric and vitaminized food.
Bibliography: e y r about S. B. Clinical forms of a chronic lymphoreticulosis, L., 1969; Kalamkaryan A. A. Clinic and therapy of reticuloses of skin, Yerevan, 1983; Pototsky I. I. Reticuloses of skin, Kiev, 1972; Skrip-kin Yu. K., etc. Guide to a children's dermatovenereology, page 88, L., 1983; Round A. T. Physiology and pathology of children of the period новорожденное™, page 206, etc., M., 1955; Brehmer - Anders-son E. Mycosis fungoides and its relation to S^zary’s syndrome, lymphomatoid papulosis and primary cutaneous Hodgkin’s disease, Acta derm. - venereol., v. 56, suppl., p. 3, 1976; N i with about 1 i s G. D. a. H e 1-wig E. B. Exfoliative dermatitis, Arch. Derm., v. 108, p. 788, 1973; Winkel-mann R. K. a. Caro W. A. Current problems in mycosis fungoides and Sezary syndrome, Ann. Rev. Med., v. 28, p.
I. M. Raznatovsky; V. A. Tabolin (ped.)*