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.

Author: Dougrel Moogutaxe
Country: El Salvador
Language: English (Spanish)
Genre: Politics
Published (Last): 17 February 2010
Pages: 218
PDF File Size: 20.18 Mb
ePub File Size: 15.99 Mb
ISBN: 874-1-53133-779-5
Downloads: 9810
Price: Free* [*Free Regsitration Required]
Uploader: Maular

National Center for Circumflfxa InformationU. Author information Article notes Copyright and License information Disclaimer. Get free access to newly published articles. References Richard G, Ringpfeil F. Cushing syndrome has been reported as a consequence of widespead application of topical corticosteroids.

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.

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

Circumflexs Dec 11; Accepted Dec Published online Dec Create a personal account to register for email alerts with links to free full-text articles. Create a free personal account to access your subscriptions, sign up for alerts, and more. Successful therapy of ichthyosis linearis circumflexa with PUVA. Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome. If you have any concerns with your skin or its treatment, see a dermatologist for advice.

Netherton’s Syndrome and Ichthyosis Linearis Circumflexa: An itchy eczematous rash may be present, especially later in childhood. J Dermatol Case Rep. Significant absorption of topical ichthyoais in 3 patients with Netherton syndrome. Microscopic examination of the hair was normal.


One of these patients had received phototherapy for eight months while curcumflexa other patient had been treated for more than four years and both had demonstrated complete response.

Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. J Eur Acad Dermatol Venerol.

Netherton syndrome is inherited as an autosomal recessive trait. Ichthyoses, Erythrokeratodermas and Related Disorders. Newborns with severe symptoms often have a poor prognosis.

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

Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. Examination of abnormal hair under microscope shows trichorrhexis invaginata defects of the hair shafts.

Discussion Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.

LEKTI is a new type of serine protease inhibitor with circumfkexa activity.

Individuals with Netherton syndrome may show some or all of these features with varying degrees of severity of their symptoms. Main observation A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.

Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results.


Netherton syndrome | DermNet NZ

Sign in to save your search Sign in to your personal account. Purchase access Subscribe to the journal. Sign in to make a comment Sign in to your personal account. Our website uses cookies to enhance your experience. A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. 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.

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

In addition, babies are more at risk of infectionwhich can be life- threatening in some cases. Other signs a patient may develop sometime throughout their lifetime include:. Get free access to newly published articles Create a personal account or sign in to: