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This case is reported because of the unusual clinical presentation and to highlight the spectrum of atypical hidradenitis suppurativa.We report a 6-year-old girl showing epidermolytic ichthyosis/epidermolytic hyperkeratosis (EI/EH). Targeted Next Generation Sequencing revealed a de novo, previously unidentified KRT1 mutation. The findings of this study expands the clinical and spectrum and genotype-phenotype correlation associated with EI/EH.Poorly controlled and long-standing hidradenitis suppurativa (HS) increases the risk of squamou
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