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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A,B Clinical improvement of skin lesions after phototherapy.

Cushing syndrome has been reported as a consequence of widespead application of topical corticosteroids. J Am Acad Dermatol. In ifhthyosis, babies are more at risk of infectionwhich can be life- threatening in some cases. Newborns with severe symptoms often have a poor prognosis.

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

Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions linearsi are bordered by a double-edged scale. Purchase access Subscribe now. Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata.

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Sign in to access your subscriptions Sign in to your personal account. Ichthyoses, Erythrokeratodermas and Related Disorders. J Eur Acad Dermatol Venerol.


A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is lineaeis by characteristic hair shaft abnormalities and atopic diathesis. A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Significant absorption of topical tacrolimus in 3 patients with Netherton syndrome.

Skin biopsy and DNA testing may also be performed to confirm diagnosis. The patient had not benefited from emollients and topical corticosteroids. Received Dec 11; Accepted Dec The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy. Sign in to make a comment Sign in to your personal account.

Clearence of ichthyosis linearis circumflexa with balneophototherapy. Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales. Our website uses cookies to enhance your experience.

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 ichthyosjs the management of this disorder. Narrowband UVB phototherapy as a novel treatment for Netherton syndrome. Netherton syndrome may increase the risk of skin cancer developing. Microscopic examination of the hair was normal. DermNet NZ does not provide an online consultation service.

Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome.

Ichthyosis linearis circumflexa

J Dermatol Case Rep. Serum biochemistries, IgE levels and complete blood count were within reference intervals. Various treatment modalities such as emmolients, keratolytics, jchthyosis, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used in the management of this disorder.


Netherton syndrome may be evident at birth or during the first weeks of life. Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Open in a separate window.

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.

Purchase access Subscribe to JN Learning for one year. Netherton syndrome is characterized by a triad of ichthyosis, hair shaft abnormalities and atopic diathesis.

linearos 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. Periods of little or no disease symptoms are interspersed with intermittent exacerbations.