AEI: annular epidermolytic ichthyosis EI: epidermolytic ichthyosis KRT: keratin PPK: palmoplantar keratoderma INTRODUCTION Annular epidermolytic ichthyosis (AEI) is a rare subtype of epidermolytic ichthyosis (EI) characterized by recurrent flares of… Click to show full abstract
AEI: annular epidermolytic ichthyosis EI: epidermolytic ichthyosis KRT: keratin PPK: palmoplantar keratoderma INTRODUCTION Annular epidermolytic ichthyosis (AEI) is a rare subtype of epidermolytic ichthyosis (EI) characterized by recurrent flares of erythematous and scaly lesions alternating with periods of almost normal skin with or without associated palmoplantar keratoderma (PPK). We report on 2 different patients with AEI showing pathogenic variants in the KRT1 and KRT10 genes respectively, and review the main clinical, histologic and molecular findings of this exceptional subset of EI.
               
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