European Journal of Dermatology


Dermoscopic and reflectance confocal microscopy features of Netherton syndrome Volume 33, issue 6, November-December 2023


  • Figure 1.
Department of Dermatology, Jiangsu Province Hospital of Chinese Medicine, Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, 210029, China

Netherton syndrome (NS) is a recessive autosomal ichthyosis arising from germline mutations in the serine protease inhibitor of the Kazal type 5 (SPINK5) gene, located on chromosome 5q31-32 [1]. NS, first described by Comel in 1949 and by Netherton in 1958 [2], has an estimated incidence of 1/200,000. It is characterized by the triad of congenital ichthyosiform erythroderma (CIE)/ichthyosis linearis circumflexa (ILC), hair shaft defects, and atopic diathesis [3]. We present a patient with NS including [...]

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