Abstract
Netherton syndrome is an autosomal recessive genodermatosis characterized by ichthyosis linearis circumflexa, trichorrhexis invaginata and atopic diathesis. The diseases is caused by mutation in the SPINK5 gene. Different therapeutic options such as topical corticosteroids, calmodulin inhibitors, psoralen, ultraviolet irradiation, retinoids and biological agents have been used in Netherton syndrome with variable success. Here we present a 6-year-old boy with a generalized erythema, lichenification, and intractable pruritus who was diagnosed with Netherton syndrome.
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References
Wilkinson RD, Curtis GH, Hawk WA. Netherton's disease: trichorrhexis invaginata (bamboo hair), congenital ichthyosiform erythroderma and the atopic diathesis. A histopathologic study. Arch Dermatol. 1964;89:46–54.
Saleem HMK, Shahid MF, Shahbaz A, Sohail A, Shahid MA, Sachmechi I. Netherton syndrome: a case report and review of literature. Cureus. 2018;10(7):e3070.
Roda Â, Mendonça-Sanches M, Travassos AR, Soares-de-Almeida L, Metze D. Infliximab therapy for Netherton syndrome: a case report. JAAD Case Rep. 2017;3(6):550–2.
Yalcin AD. A case of netherton syndrome: successful treatment with omalizumab and pulse prednisolone and its effects on cytokines and immunoglobulin levels. Immunopharmacol Immunotoxicol. 2016;38(2):162–6.
Puzenat E, et al. Congenital erythroderma should be considered as an urgent warning sign of immunodeficiency: a case of Omenn syndrome. Eur J Dermatol. 2017;27(3):313–4.
Candotti F. Clinical manifestations and pathophysiological mechanisms of the Wiskott-Aldrich syndrome. J Clin Immunol. 2018;38(1):13–27.
Park B, Liu GY. Staphylococcus aureus and hyper-IgE syndrome. Int J Mol Sci. 2020;21(23):9152.
Nawaz S, Tariq M, Ahmad I, Malik NA, Baig SM, Dahl N, Klar J. Non-bullous congentital ichthyosiform erythroderma associated with homozygosity for a novel missense mutation in an ATP binding domain of ABCA12. Eur J Dermatol. 2012;22(2):178–81.
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Hu, J., Geng, S. (2022). A 6-Year-Old Boy with a Generalized Erythema and Scales. In: Waśkiel-Burnat, A., Sadoughifar, R., Lotti, T.M., Rudnicka, L. (eds) Clinical Cases in Scalp Disorders. Clinical Cases in Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-030-93426-2_36
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DOI: https://doi.org/10.1007/978-3-030-93426-2_36
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