Darier disease (DD) is a rare autosomal dominant genodermatosis.1 Onset usually occurs during puberty following a chronic course with frequent exacerbations.1 In addition to visible and often pruritic cutaneous disease, nails and oral/ocular mucosa are commonly affected.2 Malodor and cutaneous infections (eg, widespread eczema herpeticum, Staphylococcus aureus) are frequent complications and a source of important quality-of-life impairment.2 The management of moderate to severe DD remains limited due to efficacy and/or safety considerations.3 Most commonly used strategies combine trigger avoidance, topical or systemic retinoids, and immunomodulatory agents arguing for the importance of skin barrier and inflammation in DD.3 We report 2 cases of safe and effective use of type 2 inflammation inhibitors (dupilumab and tralokinumab) in patients with severe and recalcitrant DD.