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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Received Dec 11; Accepted Dec Periods of little or no disease symptoms are interspersed with intermittent exacerbations.

A,B Clinical improvement of skin lesions after phototherapy. Our website uses cookies to enhance your experience. Newborns with severe symptoms often have a poor prognosis. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.

Introduction Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis.

Individuals with Netherton syndrome may show some or all of these features with varying degrees of severity of their symptoms. The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy. Sign in to save your search Sign in to your personal account. Additionally, phototherapy may be effective through its immunomodulatory and apoptosis inducing effects on keratinocytes, dendritic cells, mast cells and T-lymphocytes.


On the face, there was a mild, diffuse erythema with slight scaling. 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.

J Eur Acad Dermatol Venerol. Purchase access Subscribe to the journal. Elsevier Saunders Company; Author information Article notes Copyright and License information Disclaimer. Abstract Background Ichthyosis linearis circumflexa is a rare form of circumflexaa characterized by polycyclic and annular lesions which are bordered by a double-edged scale.

For infants with less severe symptoms, many will suffer from a failure to thrive in the first year of ichthyodis 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. Purchase access Subscribe to JN Learning for one year.

Ichthyosis linearis circumflexa – Wikipedia

Netherton syndrome is inherited as an autosomal recessive trait. Two patients with typical features of both ichthyosis linearis circumflexa and Netherton’s syndrome are reported. Ichthyoses, Erythrokeratodermas and Related Disorders.

The patient had not benefited from emollients and topical corticosteroids. 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 lineagis complete response. Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale.

In addition, babies are more at risk of linrariswhich can be life- icrcumflexa in some cases. 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.


Get free access to newly published articles. Successful therapy of ichthyosis linearis circumflexa with PUVA.

J Dermatol Case Rep. Skin biopsy and DNA testing may also be performed to confirm diagnosis. Linerais biochemistries, IgE levels and complete blood count were within reference intervals.

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Although these two rare variants of ichthyosis have previously circhmflexa considered to be distinct, they actually appear to be the same entity manifesting as a peculiar defect in keratinization affecting the skin and hair.

Symptoms of Netherton syndrome tend to improve with age. 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.

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

Purchase access Subscribe now. Create a free personal account to access your subscriptions, sign up for alerts, and more. The majority of lesions cleared after 30 sessions with a cumulative dose of Please review our privacy policy. Narrowband UVB phototherapy as a novel treatment for Netherton syndrome.

J Am Acad Dermatol. Biopsy revealed parakeratosis, hypogranulosis, psoriasiform acanthosis, vacuolar changes in the basal layer as well as intraepidermal neutrophilic infiltrates; mild perivascular inflammatory cell infiltration was also present in the dermis.