Secukinumab for the treatment of SAM syndrome associated with desmoglein-1 deficiency.

Treating patients with genodermatoses is challenging because of the chronic disease course and of limited available therapies. We report on the efficacy of secukinumab for the treatment of a genodermatosis caused by biallelic loss-of-function mutations in the desmoglein 1 gene (DSG1), the SAM syndrome. The SAM syndrome comprises severe dermatitis, multiple allergies and metabolic wasting and it can manifest as ichthyosiform erythroderma at birth.1 Additional variable features are palmoplantar keratoderma, hypotrichosis, recurrent skin infections, malabsorption and failure to thrive.2.