[Comèl-Netherton syndrome with bacterial superinfection]

A 10-year old boy, the child of unrelated Bosnian parents presented with a superinfected ichthyotic erythroderma. The clinical features, histological findings and hair analysis led to the diagnosis of the autosomal-recessive inherited Comel-Netherton syndrome witch bacterial superinfection. Under careful therapy with small amounts of topical tacrolimus (Protopic 0.1% ), he improved and had longer disease-free intervals. Tacrolimus was administered intermittently and not during acute flares, thus avoiding systemic resorption even after long-time treatment despite the disturbed epidermal barrier in Comel-Netherton syndrome. Staphylococcus aureus producing enterotoxin C was isolated during flares which were sometimes accompanied by marked bacterial superinfection. It is possible, that this super antigen is involved in the observed aggravation of disease. The topical therapy with tacrolimus is an easy, flexible therapeutic option for this rare genodermatosis.