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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Author information Article notes Copyright and License information Disclaimer. Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales.

Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account. If you have any concerns with your skin or its treatment, see a dermatologist for advice. Although these two rare variants of ichthyosis have previously been considered to be distinct, they actually appear to be the same entity manifesting as citcumflexa peculiar defect in keratinization affecting the skin and hair. On dermatological examination, widespread erythematous, annular, polycyclic and double-edged papulosquamous plaques were detected on the trunk and extremities [ Fig.

Netherton syndrome | DermNet NZ

Cushing syndrome has been reported as a consequence of widespead ichtnyosis of topical corticosteroids. Sign in to customize your interests Sign in to your personal account. J Dermatol Case Rep.

The term “psoriasiform ichthyosis” is proposed to include ichthyosis linearis circumflexa-Netherton’s syndrome, and perhaps other variants of ichthyosis with psoriasiform features which do not appear to fit into the existing classification of ichthyosiform dermatoses.

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. Get free access to newly published articles Create a personal account or sign in to: An itchy eczematous rash may be present, especially later in childhood. Netherton’s Syndrome and Ichthyosis Linearis Circumflexa: Other signs a patient may develop sometime throughout their lifetime include:.


There is widespread reddening erythroderma and the skin is covered in dry fine scales ichthyosis. 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.

Support Center Support Center. Purchase access Subscribe to JN Learning for one year. A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.

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.

Get free access to newly published articles. Please review our privacy policy. Significant absorption of topical tacrolimus in 3 patients with Netherton syndrome. The majority of lesions cleared after 30 sessions with a cumulative dose of Privacy Policy Terms of Use.

Sign in to save your search Sign in to your personal account. The eruption had appeared on the face as erythema and scaling at the age of one month and it had spread to the trunk and extremities during the following year. 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.

Serum biochemistries, IgE levels and complete blood count were within reference intervals. The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy. 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.


Elsevier Saunders Company; Symptoms of Netherton syndrome tend to improve with age. Self-skin examination New smartphone apps to check your skin Learn more Sponsored content. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. Purchase access Subscribe now. Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome. Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.

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

Received Dec 11; Accepted Dec Create a free personal account to access your subscriptions, sign up for alerts, and more. Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin.

Create a free personal account to download free article PDFs, sign up for alerts, and more. Oral mucosa, hair, nails, and other systemic examination were normal. Our website uses cookies to enhance your experience. Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results.

Purchase access Subscribe to the journal. LEKTI is a new type of serine protease inhibitor with antitrypsin activity. Periods of little or no disease symptoms are interspersed with intermittent exacerbations.

Narrowband UVB phototherapy as a novel treatment for Netherton syndrome.