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The syndrome of Neterton: causes, symptoms, diagnosis, treatment
Last reviewed: 18.10.2021
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EV Netterton (1958) described the combination of ichthyosis - ichthyosiform congenital erythroderma (lamellar ichthyosis) with hair lesion in the form of nodular trichorexis in combination with atopy.
What causes the Netherton syndrome?
Previously believed that the disease is contagious. Currently, an autosomal recessive type of inheritance is assumed. Mostly women are ill.
Histopathology
Characteristic are proliferative hyperkeratosis (sometimes with parakeratosis), acanthosis with spongiosis, normal or thickened granular layer, hypertrophy of dermal papillae, increased sebaceous and sweat glands, edema and mild chronic inflammatory perivascular cataracts in the upper parts of the dermis, consisting of lymphocytes and plasma cells.
Symptoms of the Netherton syndrome
At the birth of the child, the skin of the fetus is completely covered with a dry dry yellowish brown film resembling a collodion. In the literature, the description of the "colloidal" fetus (syn "glossy baby") belongs to one of the severe manifestations of the disease. In some cases, the collodion film, having existed for a while, turns into large flakes (lamellar desquamation of newborns) and even completely disappears in early infancy, the skin remains normal throughout life. In most cases, the scales formed from the film remain on the skin for life (lamellar ichthyosis).
With age, erythroderma regresses, and hyperkeratosis intensifies. The lesion seizes all skin folds, and the skin changes in them are often more pronounced. The skin of the face is usually red, stretched, scaly.
The hairy part of the head is covered with abundant scales, the ears are twisted, with abundant horny layers, including in the auditory canals. There is increased sweating of the skin of the palms, soles, faces, rapid growth of hair and nails (hyperdermotomy).
Nail plates are deformed, thicken; marked subungual hyperkeratosis, diffuse keratosis of the palms and soles. A characteristic symptom of lamellar ichthyosis is also the ectropion, which is often accompanied by lagophthalmus, keratitis, photophobia. Sometimes, with lamellar ichthyosis, mental retardation is noted.
Structural abnormalities of hair in the syndrome of Netherton can be of different types: twisted hair; invaginating trichorexis - transverse ruptures of hair with vkolachivaniem of the proximal segment into the distal; knotty trichorexis - structural deformation of hair with the formation of knots by the type of thickening on bamboo rods. Changes in thickness, hair are observed in the syndrome of Netteron by combining lamellar ichthyosis with splitting of the trunk of the hair due to cuticle damage on 3-10 branches (trichotypes), while the hair does not reach a length of more than 5 cm. The most common pathology of hair with the syndrome of Netherton is trichorexis or invaginated). As a result of dystrophic changes in the hair, focal or subtotal alopecia can develop.
What's bothering you?
How to recognize the syndrome of Netherton?
The syndrome of Neterton is differentiated with desquamative erythroderma Leiner-Moussa, psoriatic erythroderma, Ritter's exfoliative dermatitis, bullous epidermolysis.
What do need to examine?
How to examine?
Treatment of the Netherton syndrome
Therapeutic measures are carried out both with ichthyosis.