Erythroderma in a neonate

• Type of Material: Article
• Authors: Kristin N Smith, University of Florida; Elizabeth L Bisbee, University of Florida; Penelope J Kallis, University of Florida; Kerrie Satcher, Emory University; Jennifer Schoch, University of Florida; Sylvie Fraitag, Hôpital Necker Enfants Malades; Vladimir Vincek, University of Florida; Kiran Motaparthi, University of Florida C
• Language: English
• Date: 2022-03-01
• Publisher: Elsevier, Inc
• Publication Version: Final Published Version
• Copyright Statement: © 2022 by the American Academy of Dermatology, Inc. Published by Elsevier, Inc.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.jdcr.2021.09.047
• Title of Journal or Parent Work: JAAD Case Reports
• Volume: 21
• Start Page: 97
• End Page: 100
• Grant/Funding Information: None
• Keywords: immunohistochemistry; secukinumab; Netherton syndrome; IL, interleukin; interleukin-17; LEKTI, lymphoepithelial Kazal-type–related inhibitor; exfoliative; NS, Netherton syndrome; dermatitis
• Research Categories: Health Sciences, Pathology

Abstract

A newborn boy with no family history of erythroderma or ichthyosis presented with fine, superficial scale overlying diffuse mild erythema (Fig 1, A). At 5 weeks of age, he demonstrated worsening erythroderma with superficial desquamation (Fig 1, B). A complete blood cell count with differential count showed an elevated level of eosinophils. A punch biopsy was performed that demonstrated psoriasiform hyperplasia of the epidermis with enlarged nuclei in the stratum corneum and almost complete absence of the stratum granulosum. Immunostaining for lymphoepithelial Kazal-type–related inhibitor (LEKTI) was performed and showed the absence of staining in the epidermis and the epithelium of hair follicles (Figs 2 and ​and3)3) compared with the control (Fig 4).