TOURAINE POLYKERATOSIS

From Big Medical Encyclopedia

TOURAINE POLYKERATOSIS (A. Tou-raine, fr. dermatologist, 1883 — 1961; Greek poly is a lot of + keras, ke-ratos a horn, horn substance + - osis; synonym: an inborn polykeratosis, a polydisplasia of an ectoderm) — group of the hereditary inborn nevoid syndromes which are characterized by different keratoza which are combined with other displays of a dysplasia and dystrophy of skin and its appendages (nails, hair, teeth, sweat and sebaceous glands), and also anomalies of development nervous and other systems of an organism.

In 1942 Touraine allocated this group of syndromes. It included in it the anomalies of an epidermopoiesis which are characterized by the wrong keratinization and bulging. I. I. Pototsky (1977) carries Other to ektomezodermalny displaziya.

The most continuous manifestation Other are palmar and bottom keratoza (see), is more often than diffusion character with cracks and periodically arising bubbles. The keratosis is often combined with a local palmar and bottom hyperhidrosis (see), the general hypohydrosis (see Angidrotichesky syndromes), a pachyonychia (a thickening of nail plates) or an onychogryphosis (nails, similar to claws of birds), a xeroderma of a trunk and extremities, an ikhtioziformny erythrosis (see the Ichthyosis), a White's disease, and also a hypotrichosis, a hypoplasia and dystrophy of teeth and other anomalies. Changes of bones are more often shown by an osteochondritis (see) bones of knee, hip and other joints.

At separate syndromes Other are observed, in addition to keratotichesky, and other changes of skin — a poikiloderma (see), bubbles (see the Epidermolysis violent) — and mucous membranes — a leukoplakia (see), a keratitis (see), a cataract (see). Disturbances of mentality are possible.

Conditionally carry such independent nosological forms as Engmen's syndrome to the Other group — Cole (see the Poikiloderma), Yadasson's syndrome — Lewandowski, and also Schaefer's syndrome and a syndrome of Siemens, to-rye are clinically close among themselves and to Yadasson's syndrome — Lewandowski (see Yadasson — - Lewandowski a syndrome).

The diagnosis Other is made on the basis by a wedge, pictures, in some cases carry out gistol. a research, at the same time changes in the centers of a keratosis are characterized by a hyperkeratosis, granulezy, an acanthosis, are possible a dyskeratosis, lymphoid gistiotsitar-ny infiltrate around vessels of a derma, akantokeratoliz.

Symptomatic treatment. Appoint vitamins — Aevitum, B6, B12, and also vasodilators (komplamin, Nospanum), etc. Outwardly apply keratolytic ointments on sites of a keratodermia.

The forecast depends on extent of defeat of a nervous system, bones, etc.

Prevention is performed preferential in the course of medicogenetic consultation (see. Medicogenetic consultation). At the same time consider the frequency of pathology, type of its inheritance, making the forecast about a possibility of its manifestation at succeeding generation in a specific family tree.

Bibliography: Babayants R. S. To vop

dew about Touraine's polykeratosis, Vestn. dermas, and veins., No. 6, page 13, 1959; D yu and V. M. and Corsoun V. F. Poliqueratoz Tourena, in the same place, No. 8, page 59, 1982; P about p x r and - with t about in the Item. Skin diseases at children's age, the lane with bolg., page 683, Sofia, 1963; Pototsky I. And, Giperkeratoza, Kiev, 1977; T about u r an i n e A. L’ery-th^me palmo-piantaire hei45ditaire (&description 6tude gi^tiquej, Ann. Derm. Syph. (Paris), t. 2, p. 119, 1942.

S. S. Kryazheva.

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