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We describe a case of generalized inflammatory PSS in an infant, presenting at day two of life with ichthyosiform erythroderma and superficial peeling of the skin. Hair microscopy showed trichorrhexis invaginata. Normal amounts of skin LEKT1, a product of <jats:italic>SPINK5<\/jats:italic> on immunohistochemical staining excluded a diagnosis of Netherton syndrome. Genetic analysis revealed a homozygous novel complete <jats:italic>CDSN<\/jats:italic> deletion, estimated 4.6\u00a0kb in size, supporting the diagnosis of generalized inflammatory PSS.<\/jats:p>","DOI":"10.1111\/pde.14939","type":"journal-article","created":{"date-parts":[[2022,2,18]],"date-time":"2022-02-18T05:58:40Z","timestamp":1645163920000},"page":"268-272","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A novel pathogenic variant in the corneodesmosin gene causing generalized inflammatory peeling skin syndrome with marked eosinophilia and trichorrhexis invaginata"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7679-5207","authenticated-orcid":false,"given":"Helen","family":"Gordon","sequence":"first","affiliation":[{"name":"Department of Dermatology Starship Children's Health Auckland District Health Board  Auckland New 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