Ýktiyozis linearis sirkumfleksadan netherton sendromuna deðiþen klinik spektrum: 3 olgu

ýktiyozis linearis sirkumfleksa eritemli, çift-kenarlý skuamlý lezyonlarla karakterize nadir bir iktiyoziform dermatozdur. Netherton sendromu ise; genellikle iktiyozis linearis sirkumfleksa þeklinde kendini gösteren iktiyoziform dermatit, kýl gövdesinde çeþitli bozukluklar ve atopik diyatezden meydana gelen klasik bir triad olarak tanýmlanmýþtýr. Burada Netherton sendromlu bir olgu ve atopik diyatez bulgularýnýn eþlik ettiði iki iktiyozis linearis sirkumfleksa olgusu sunulmaktadýr. ýktiyozis linearis sirkumfleksaya atopi bulgularýnýn eþlik edebileceði ve Netherton sendromlu olgularýn saç bulgularýnda spontan düzelmeler olabileceði için, bu iki dermatozun ayný hastalýk spektrumun iki ucu olarak deðerlendirilebileceðini düþünüyoruz.

The clinical spectrum varying from ichthyosis linearis circumflexa to Netherton syndrome: 3 cases

Ichthyosis linearis circumflexa is a rare ichthyosiform dermatosis characterized by erythmatous, double-edged scaling lesions. Netherton syndrome is defined as a classical triad composed of ichthyosiform dermatosis presenting mostly as ichthyosis linearis circumflexa, various hair shaft defects and atopic manifestations. We report 2 cases of ichthyosis linearis circumflexa associated with some atopic manifestations and 1 case of Netherton syndrome. Since atopic manifestations may be associated with ichthyosis linearis circumflexa and spontaneous remission may be observed in hair abnormalities in Netherton syndrome, we think that these two dermatoses might be regarded as two ends of the same disease spectrum.