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Neterton's syndrome


Neterton's syndrome (Е. W. Netherton, the modern American dermatologist) — a hereditary combination of an inborn ikhtioziformny erythrosis (a skin pokrasneiiya), sites of a thickening of hair at the expense of invagination (as if insertions or a vvorachivaniye) their distal (lower) parts in proximal (upper), atopic dermatitis and a cataract; sometimes is followed by an aminoaciduria (abnormal composition of urine); occurs only at women.

Reasons of a syndrome of Neterton:

Earlier considered that the disease is infectious. Now the autosomal and recessive mode of inheritance is supposed. Women are ill generally.

Symptoms of a syndrome of Neterton:

At the child's birth skin of a fruit is completely covered with the thin dry yellowish-brown film reminding a collodion. In literature the description of a "colloid" fruit (Xing. "the glossy child") belongs to one of heavy displays of a disease. In some cases a collodion film, having existed some time, turns into large scales (lamellar desquamation of newborns) and at early chest age completely disappears, skin throughout all life remains normal. In most cases the scales which are formed of a film remain on skin for life (a lamellar ichthyosis).

With age the erythrosis regresses, and the hyperkeratosis amplifies. Defeat takes all skin folds, and the changes of skin in them which were often more expressed. Face skin is usually red, tense, it is shelled.

Pilar part of the head is covered with plentiful scales, the auricles twisted with plentiful horn stratifications including in acoustical passes. The increased perspiration of skin of palms, soles, persons is observed, rapid growth of hair and nails is noted (giperdermotogofiya).

Nail plates are deformed, thickened; the hyponychial hyperkeratosis, a diffusion keratosis of palms and soles is noted. A characteristic symptom of a lamellar ichthyosis is also ectropion which is quite often accompanied a lagophthalmia, a keratitis, photophobia. Sometimes at a lamellar ichthyosis mental retardation is noted.

Structural anomalies of hair at Neterton's syndrome can be different types: the overwound hair; the invaginating trichorrhexis - cross ruptures of hair with a vkolachivaniye of a proximal piece in distal; a knotty trichorrhexis - structural deformation of hair with formation of nodes as thickenings on bamboo cores. Changes of thickness, a hair are observed at Neterton's syndrome by a combination of a lamellar ichthyosis to splitting of a trunk of hair due to disturbance of a cuticle on 3-10 branches (трихоптилез), at the same time hair do not reach length more than 5 cm. The most often found pathology of hair at Neterton's syndrome is the trichorrhexis (knotty or invaginating). As a result of dystrophic changes of hair the focal or subtotal alopecia can develop.

Treatment of a syndrome of Neterton:

Medical events as are held at an ichthyosis.

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