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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Get free access to newly published articles Create a personal account or sign in to: Periods of little or no disease symptoms are interspersed with intermittent exacerbations. Other signs a patient may develop sometime throughout their lifetime include:. Netherton syndrome may increase the risk of skin cancer developing.

Netherton syndrome | DermNet NZ

The majority of lesions cleared after 30 sessions with a cumulative dose of There is widespread reddening erythroderma and the skin is covered in dry fine scales ichthyosis.

NB UVB phototherapy can be considered a rational approach with regard to the side effects of topical therapies and the variable results obtained from systemic retinoids in the management of this disorder.

Skin biopsy and DNA testing may also be performed to confirm diagnosis. Sign in to make a comment Sign in to your personal account.

Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata. Netherton syndrome should be at the top of the differential diagnosis list in a newborn with erythroderma and abnormal-looking scalp hairor in an older child with ichthyosis linearis circumflexa and sparse lustreless hair.

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Netherton syndrome

If you have any concerns with your skin or its treatment, see a dermatologist for advice. Ichthyoses, Erythrokeratodermas and Related Disorders. A diagnosis of ichthyosis linearis circumflexa was established based on circumflesa clinical and histopathological findings. Discussion Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.

A,B Widespread, erythematous, polycyclic, scaly eruption with double-edged scales.

A,B Clinical improvement of skin lesions after phototherapy. Two patients with typical features of both ichthyosis linearis circumflexa and Netherton’s syndrome are reported. Create a free personal account to download free article PDFs, sign up for alerts, and more. National Center for Biotechnology InformationU. Open in a separate window. 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’s Syndrome and Ichthyosis Linearis Circumflexa: Significant improvement was observed after amild flare-up which ocurred at the 20th session [ Fig. Cushing syndrome has been reported as a consequence of widespead application of topical corticosteroids.

DermNet NZ does not provide an online consultation service. Create a personal account to register for email alerts with links to free full-text articles. This does icchthyosis usually develop until after 2 years of age but may occur earlier.

Ichthyosis Linearis Circumflexa as the Only Clinical Manifestation of Netherton Syndrome.

Author information Article notes Copyright and License information Disclaimer. Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Microscopic examination of the hair was normal. His brother was healthy, the parents were nonconsanguineous and there was no personal or family history of atopy. Received Dec 11; Accepted Dec Main observation A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.


Successful therapy of ichthyosis linearis circumflexa with PUVA. There is no specific treatment for Netherton syndrome. 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.

Individuals with Netherton syndrome may show some or all of these features with varying degrees of severity of their symptoms.

Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales.

Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. LEKTI is a new type of serine protease inhibitor with antitrypsin activity.

The exact mechanism of action of phototherapy in this condition is unknown, but it has been proposed that phototherapy may enhance the synthesis of other serine protease inhibitors that compensate the activity of the defective LEKTI function. The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy.