Papillon-Lefèvre syndrome with excellent response to risankizumab

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive genodermatosis. It is clinically characterized by diffuse palmoplantar keratoderma (PPK), psoriasiform skin lesions, and rapidly progressive periodontopathy. Management of PLS can be challenging. Herein, we present the case of a 27-year-old female who experienced poor response to multiple therapies, including topical keratolytic creams, oral isotretinoin and acitretin, and the tumor necrosis factor (TNF) inhibitor adalimumab. Notably, she achieved complete resolution of her cutaneous manifestations following treatment with the interleukin (IL)-23 inhibitor risankizumab.