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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Other signs a patient may develop sometime throughout their lifetime include:. The patient had not benefited from emollients and topical corticosteroids.
Netherton syndrome | DermNet NZ
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. 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.
Published online Dec Purchase access Subscribe to JN Learning for one year. Ichhyosis free access to newly published articles Create a personal account or sign in to: A,B Widespread, erythematous, polycyclic, scaly eruption with double-edged scales.
Ichthyosis Linearis Circumflexa as the Only Clinical Manifestation of Netherton Syndrome.
The majority of lesions cleared after 30 sessions with a cumulative dose of Although these two rare variants of ichthyosis have previously been considered to be distinct, they actually appear to be the same entity manifesting as a peculiar defect in keratinization affecting the skin and hair. Discussion Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.
Netherton syndrome may increase the risk of skin cancer developing. Serum biochemistries, IgE levels and complete blood count were within reference intervals. There is widespread reddening erythroderma and the ccircumflexa is covered in dry fine scales ichthyosis. Ichthyoses, Erythrokeratodermas and Related Disorders.
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. Author information Article notes Copyright and License information Disclaimer. Netherton syndrome is characterized by a triad of ichthyosis, hair shaft abnormalities and atopic diathesis.
Additionally, phototherapy may be effective through its immunomodulatory and apoptosis inducing effects on keratinocytes, dendritic cells, mast cells and T-lymphocytes.
A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Individuals with Netherton syndrome may show some or all of these features with varying degrees of severity of their symptoms.
Clearence of ichthyosis linearis circumflexa with balneophototherapy. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account. Various treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used in the management of this disorder.
This does not usually develop until after 2 years of age but may occur earlier.
Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.
Altman J, Stroud J. Two patients with typical features of both ichthyosis linearis circumflexa and Netherton’s syndrome are reported.
Netherton syndrome should be at the top of the differential diagnosis list in a newborn with erythroderma and abnormal-looking scalp lihearisor in an older child with ichthyosis linearis circumflexa and sparse lustreless hair. References Richard G, Ringpfeil F.
Ichthyosis linearis circumflexa – Wikipedia
Main observation A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions. Abstract Background Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale.
If you have any concerns ichthyoiss your skin or its treatment, see a dermatologist for advice.
One of these patients had received phototherapy for eight months while the other patient had been treated for more than four years and both had demonstrated complete response. Examination of abnormal hair under microscope shows trichorrhexis invaginata defects of the hair shafts. Ichthysois Eur Acad Dermatol Venerol. Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.