Successful Treatment of Inflammatory Dermatitis Associated With ALOXE3 Nonsyndromic Epidermal Differentiation Disorder Using Dupilumab

Dupilumab has emerged as a promising treatment for inflammatory dermatitis in some epidermal differentiation disorders (EDDs, formerly known as ichthyosis). We report a 5-month-old female with recurrent generalized skin peeling and erythematous plaques since birth that were refractory to multiple topical therapies; she was diagnosed with ALOXE3-associated nonsyndromic epidermal differentiation disorder (ALOXE3-nEDD) by whole exome sequencing. Following initiation of dupilumab, she experienced near-complete resolution of erythematous plaques within 9 months and improvement of pruritus, with sustained response over 2 years of treatment. This case highlights dupilumab as a potential treatment for inflammatory dermatitis associated with ALOXE3-nEDD and similar EDDs.