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

[摘要] Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis. Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results. Topical therapies may result in considerable absorption because of the defective barrier function in ichthyosis linearis circumflexa/Netherton syndrome.

[学科分类] 皮肤病学

[关键词] ichthyosis;Netherton syndrome;phototherapy;treatment;trichorrhexis invaginata;UV