July 11, 2020

Ichthyosis linearis circumflexa is a distinctive skin condition of generalized hyperkeratosis and polycyclic and serpiginous erythematous plaques with a. It manifests as collodion baby at birth and refractory erythroderma during infancy and early childhood days. Ichthyosis linearis circumflexa (ILC). A 7 years old girl, born of a non-consanguineous marriage, presented with multiple asymptomatic dry and scaly patches over her body since infancy. The.

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Netherton syndrome is a rare inherited disorder that presents with the three following characteristics:. Individuals with Netherton syndrome may show some or all of these features with varying degrees of severity of their symptoms.

Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.

Netherton syndrome may be evident at birth or during the first weeks of life. There is widespread reddening erythroderma and the skin is covered in dry fine scales ichthyosis.

An itchy eczematous rash may be present, especially later in childhood. In addition, babies are more at risk of infectionwhich can be life- threatening in some cases. Newborns with severe symptoms often have a poor prognosis. For infants with less severe symptoms, many will suffer from a failure to thrive in the first year of life and by the second year of life although the health of most children will start to improve, most will remain underweight and of short stature.


Other signs a patient may develop sometime throughout their lifetime include:.

Ichthyosis linearis circumflexa – Wikipedia

Netherton syndrome is inherited as an autosomal recessive trait. In some cases there is no family history of the trait and Netherton syndrome is revealed when two unaffected parents who are both carriers of the mutated recessive gene have a child who receives both copies of the recessive gene.

Netherton syndrome should be at the top of the differential diagnosis list in a newborn with erythroderma ichthyoxis abnormal-looking scalp hairor in an older child with ichthyosis linearis circumflexa and sparse lustreless circumfflexa.

Examination of abnormal hair under microscope shows trichorrhexis invaginata defects of the hair shafts. This does not usually develop until after 2 years of age but may occur earlier. Skin biopsy and Circjmflexa testing may also be performed to confirm diagnosis.

There is no specific treatment for Netherton syndrome. The goals of treatment are to manage the symptoms circkmflexa prevent skin infections and other complications.


Netherton syndrome

Symptoms of Netherton syndrome tend to improve with age. Periods of little or no disease symptoms are interspersed with intermittent exacerbations.

Netherton syndrome may increase the risk of skin cancer developing.

Self-skin examination New smartphone apps to check your skin Learn more Sponsored content. DermNet NZ does not provide an online consultation service. If you have any concerns with your skin or its treatment, see a dermatologist for advice.