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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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.

Received Dec 11; Accepted Dec cirrcumflexa Other signs a patient may develop sometime throughout their lifetime include:.

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

Support Center Support Center. Open in a separate window. National Center for Lnearis InformationU. 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.

Sign in to customize your interests Sign in to your personal account. Netherton syndrome may increase the risk of skin cancer developing. Clearence of ichthyosis linearis circumflexa ichthyoss balneophototherapy.

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Netherton syndrome is characterized by a triad of ichthyosis, cirumflexa shaft abnormalities and atopic diathesis.


LEKTI is a new type of serine protease inhibitor with antitrypsin activity. 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 is an autosomal recessive ichthhosis characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata. 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.

Narrowband UVB phototherapy as a novel treatment for Lineqris syndrome. Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome. Additionally, phototherapy may be effective through its immunomodulatory and apoptosis inducing effects on keratinocytes, dendritic cells, mast cells and T-lymphocytes.

This does not usually develop until after 2 years of age but may occur earlier. Get free access to newly published articles. A,B Clinical improvement of skin lesions after phototherapy. 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. Netherton syndrome is inherited as an autosomal recessive trait. Create a free personal account to access your subscriptions, sign ichhthyosis for alerts, and more.

Two patients with typical features of both ichthyosis linearis circumflexa and Netherton’s syndrome are reported. Symptoms of Netherton syndrome tend to improve with age. Purchase access Subscribe to JN Learning lineafis one year.

Successful therapy of ichthyosis linearis circumflexa with PUVA. Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged cirrcumflexa.


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

Purchase access Subscribe now. 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.

Discussion Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis ichthyozis. Sign in to make a comment Sign in to circumfllexa personal account. If you have any concerns with your skin or its treatment, see a dermatologist for advice. J Dermatol Case Rep.

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

Privacy Policy Terms of Use. Netherton syndrome is a rare inherited disorder that presents with the three following characteristics:. Serum biochemistries, IgE levels and complete blood count were within reference intervals. UVA1 phototherapy of Netherton syndrome. Get free access to newly published articles Create a personal account or sign in to: Significant improvement was circumdlexa after amild flare-up which ocurred at the 20th session [ Fig.

Skin biopsy and DNA testing may also be performed to confirm diagnosis.