Netherton syndrome: effect on ichthyosis linearis circumflexa with dupilumab

To evaluate the efficacy of Dupilumab in treating Netherton Syndrome (SPINK5-sEDD).

We present a girl with SPINK5-sEDD who failed topical therapy. Treatment with Dupilumab 300mg every 4 weeks was initiated.

Expeditious response to Dupilumab was demonstrated in terms of eczema, itch and hair condition, as evidenced by improvement in the Scoring of Atopic Dermatitis (SCORAD), the Eczema Area and Severity Index (EASI) and the Children's Dermatology Life Quality Index (CDLQI) by more than 40%. However, she continued to experience significant recurrent flares of ichthyosis linearis circumflexa (ILC).

Our case illustrates promising response to dupilumab in terms of eczema and hair growth, as well as a significant drop in serum IgE level, but ichthyosis linearis circumflexa persists.