The effect of Gladskin (Staphefekt) treatment on the severity of skin symptoms in patients with Netherton syndrome

Netherton syndrome is a rare autosomal recessive form of severe ichthyosis caused by mutations in the serine protease inhibitor Kazal type 5 (SPINK5). The disease is characterized by ichthyosis linearis circumflexa, erythematous, dry and scaling skin lesions typical for NS. Colonization of the skin and mucosa with Staphylococcus aureus might worsen the skin symptoms of NS and causes recurrent skin infections in these patients. Antibiotics are often used to treat these skin symptoms and infections. Because of common side effects and increasing multidrug resistance of S. aureus, NS patients might benefit from alternative treatment. Gladskin is a product for topical use, the proprietary enzyme in the Gladskin products is called Staphefekt SA.100 (Staphefekt). Staphefekt is an endolysin that specifically lyses the cell membrane of S. aureus. In vitro results showed that Staphefekt kills S. aureus, including methicillin resistant S. aureus. Treatment with Gladskin might decrease S. aureus on the skin and consequently decrease symptoms and severity of S. aureus related disease.

- A decrease in Staphylococcus aureus colonization on skin

- A decrease in skin symptoms (including erythema, pain, desquamations, pruritis infections)

TO (week 0): enrollment

T1 (week 2) : start of intervention

T2 (week 10): end of intervention

The intervention includes Gladskin (Staphefekt) gel application