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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Netherton syndrome | DermNet NZ
Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales. Open in a separate window.
Additionally, phototherapy may be effective through its immunomodulatory and apoptosis inducing effects on keratinocytes, dendritic cells, mast cells and T-lymphocytes. UVA1 phototherapy of Netherton syndrome. Narrowband UVB phototherapy as a novel treatment for Netherton syndrome. Create a free personal account to access your subscriptions, sign up for alerts, and more. Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale.
Case Report A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin.
Sign in to access your subscriptions Sign in to your personal account. Self-skin examination New smartphone apps to check your skin Learn more Sponsored content. 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.
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 circumflexaa year. 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 circkmflexa this disorder. The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy.
Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis.
Successful therapy of ichthyosis linearis circumflexa with PUVA. This does not usually develop until after 2 years of age but may occur earlier.
Netherton syndrome may be evident at birth or during the first weeks of life. 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 characterized by a triad of ichthyosis, hair shaft abnormalities and atopic diathesis. 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.
Purchase access Subscribe now. A year-old Caucasian male presented for evaluation of a long standing skin condition characterized by dry, red and scaling skin. Introduction Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales.
Discussion Netherton syndrome is an autosomal recessive disorder characterized by ichthyosis linearis circumflexa, atopic diathesis and hair shaft abnormalities such as trichorrhexis invaginata. Other signs a patient may develop sometime throughout their lifetime include:.
Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. Various treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used cirucmflexa the management of this disorder. Create a personal account to register for email alerts with links to free full-text articles.
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. His brother was healthy, the parents were nonconsanguineous and there was no personal or family history of atopy. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.
Received Dec 11; Accepted Dec Main observation A year-old patient diagnosed as ichthyosis linearis ichhhyosis demonstrated circumf,exa improvement with narrowband UVB phototherapy at the end of 30 sessions.